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Source: http://www.doksinet Effective for Maintenance of Certification examinations administered through March 2018. T HE A MERICAN B OARD OF P EDIATRICS ® CONTENT OUTLINE Pediatric Rheumatology Maintenance of Certification (MOC) Examinations Source: http://www.doksinet INTRODUCTION This document was prepared by the American Board of Pediatrics Subboard of Pediatric Rheumatology for the purpose of developing in-training, certification, and maintenance of certification examinations. The outline defines the body of knowledge from which the Subboard samples to prepare its examinations. The content specification statements located under each category of the outline are used by item writers to develop questions for the examinations; they broadly address the specific elements of knowledge within each section of the outline. Source: http://www.doksinet EXAM BLUEPRINT Pediatric Rheumatology Each Pediatric Rheumatology exam is built to the same specifications, also known as the

blueprint. This blueprint is used to ensure that, for the initial certification and in-training exams, each exam measures the same depth and breadth of content knowledge. Similarly, the blueprint ensures that the same is true for each Maintenance of Certification exam form. The table below shows the percentage of questions from each of the content domains that will appear on an exam. Please note that the percentages are approximate; actual content may vary. Content Categories Initial Certification and In-Training Maintenance of Certification (MOC) 1. Core Knowledge in Scholarly Activities 5% 4% 2. Etiology and Pathogenesis 8% 7% 3. Drug Therapy 10% 12% 4. Musculoskeletal Pain Syndromes of Nonrheumatic Origin 4% 4% 5. JRA and Juvenile Idiopathic Arthritis (JIA) 12% 12% 6. Spondyloarthropathies 7% 7% 7. Systemic Lupus Erythematosus 11% 11% 8. Juvenile Dermatomyositis 6.5% 6.5% 9. Vasculitis 7% 7% 10. The Sclerodermas and Related Disorders 5% 5%

11. Miscellaneous Rheumatologic Diseases 7% 7% 12. Immunodeficiencies and the Rheumatic Diseases 2% 2% 13. Arthritis Related to Infection 3% 3% 14. Skeletal Malignancies and Related Disorders 1.5% 1.5% 15. Bone and Connective Tissue Disorders 4% 4% 16. Musculoskeletal Manifestations of Other Chronic Diseases 3% 3% 17. Rehabilitation 2% 2% 18. Psychosocial Issues/Developmental Issues/ Chronic Illness 2% 2% Source: http://www.doksinet Rheumatology I. A. Core Knowledge in Scholarly Activities Principles of Use of Biostatistics in Research 1. Types of variables a. Distinguish types of variables (eg, continuous, categorical, ordinal, nominal) b. Understand how the type of variable (eg, continuous, categorical, nominal) affects the choice of statistical test 2. Distribution of data a. Understand how distribution of data affects the choice of statistical test b. Differentiate normal from skewed distribution of data c. Understand the appropriate use of the

mean, median, and mode d. Understand the appropriate use of standard deviation e. Understand the appropriate use of standard error 3. Hypothesis testing a. Distinguish the null hypothesis from an alternative hypothesis b. Interpret the results of hypothesis testing 4. Statistical tests a. Understand the appropriate use of the chi-square test versus a t-test b. Understand the appropriate use of analysis of variance (ANOVA) c. Understand the appropriate use of parametric (eg, t-test, ANOVA) versus non-parametric (eg, Mann-Whitney U, Wilcoxon) statistical tests d. Interpret the results of chi-square tests e. Interpret the results of t-tests f. Understand the appropriate use of a paired and non-paired t-test g. Determine the appropriate use of a 1- versus 2-tailed test of significance h. Interpret a p-value i. Interpret a p-value when multiple comparisons have been made j. Interpret a confidence interval k. Identify a type I error l. Identify a type II error 5. Measurement of association

a. Differentiate relative risk reduction from absolute risk reduction b. Calculate and interpret a relative risk c. Calculate and interpret an odds ratio d. Interpret a hazard ratio e. Understand the uses and limitations of a correlation coefficient 6. Regression a. Identify when to apply regression analysis (eg, linear, logistic) b. Interpret a regression analysis (eg, linear, logistic) c. Identify when to apply survival analysis (eg, Kaplan-Meier) d. Interpret a survival analysis (eg, Kaplan-Meier) 7. Diagnostic tests Source: http://www.doksinet a. Recognize the importance of an independent "gold standard" in evaluating a diagnostic test b. Calculate and interpret sensitivity and specificity c. Calculate and interpret positive and negative predictive values d. Understand how disease prevalence affects the positive and negative predictive value of a test e. Calculate and interpret likelihood ratios f. Interpret a receiver operator characteristic curve g. Interpret and

apply a clinical prediction rule 8. Systematic reviews and meta-analysis a. Understand the purpose of a systematic review b. Understand the advantages of adding a meta-analysis to a systematic review c. Interpret the results of a meta-analysis d. Identify the limitations of a systematic review e. Identify the limitations of a meta-analysis B. Principles of Epidemiology and Clinical Research Design 1. Study types a. Distinguish between Phase I, II, III, and IV clinical trials b. Recognize a retrospective study c. Understand the strengths and limitations of retrospective studies d. Recognize a case series e. Understand the strengths and limitations of case series f. Recognize a cross-sectional study g. Understand the strengths and limitations of cross-sectional studies h. Recognize a case-control study i. Understand the strengths and limitations of case-control studies j. Recognize a longitudinal study k. Understand the strengths and limitations of longitudinal studies l. Recognize a

cohort study m. Understand the strengths and limitations of cohort studies n. Recognize a randomized-controlled study o. Understand the strengths and limitations of randomized-controlled studies p. Recognize a before-after study q. Understand the strengths and limitations of before-after studies r. Recognize a crossover study s. Understand the strengths and limitations of crossover studies t. Recognize an open-label study u. Understand the strengths and limitations of open-label studies v. Recognize a post-hoc analysis w. Understand the strengths and limitations of post-hoc analyses x. Recognize a subgroup analysis y. Understand the strengths and limitations of subgroup analyses 2. Bias and confounding a. Understand how bias affects the validity of results b. Understand how confounding affects the validity of results c. Identify common strategies in study design to avoid or reduce bias Source: http://www.doksinet d. e. Identify common strategies in study design to avoid or reduce

confounding Understand how study results may differ between distinct sub- populations (effect modification) 3. Causation a. Understand the difference between association and causation b. Identify factors that strengthen causal inference in observational studies (eg, temporal sequence, dose response, repetition in a different population, consistency with other studies, biologic plausibility) 4. Incidence and prevalence a. Distinguish disease incidence from disease prevalence 5. Screening a. Understand factors that affect the rationale for screening for a condition or disease (eg, prevalence, test accuracy, risk-benefit, disease burden, presence of a presymptomatic state) 6. Decision analysis a. Understand the strengths and limitations of decision analyses b. Interpret a decision analysis 7. Cost-benefit, cost-effectiveness, and outcomes a. Differentiate cost-benefit from cost-effectiveness analysis b. Understand how quality-adjusted life years are used in cost analyses c. Understand the

multiple perspectives (eg, of an individual, payor, society) that influence interpretation of cost-benefit and cost- effectiveness analyses 8. Sensitivity analysis a. Understand the strengths and limitations of sensitivity analysis b. Interpret the results of sensitivity analysis 9. Measurement a. Understand the types of validity that relate to measurement (eg, face, construct, criterion, predictive, content) b. Distinguish validity from reliability c. Distinguish internal from external validity d. Distinguish accuracy from precision e. Interpret measurements of interobserver reliability (eg, kappa) f. Understand and interpret Cronbachs alpha C. Applying Research to Clinical Practice 1. Assessment of study design, performance & analysis (internal validity) a. Recognize when appropriate control groups have been selected for a case-control study b. Recognize when appropriate control groups have been selected for a cohort study c. Recognize the use and limitations of surrogate

endpoints d. Understand the use of intent-to-treat analysis e. Understand how sample size affects the power of a study f. Understand how sample size may limit the ability to detect adverse events g. Understand how to calculate an adequate sample size for a controlled trial (eg, clinically meaningful difference, variability in measurement, choice of alpha and beta) 2. Assessment of generalizability (external validity) Source: http://www.doksinet a. b. c. Identify factors that contribute to or jeopardize generalizability Understand how non-representative samples can bias results Assess how the data source (eg, diaries, billing data, discharge diagnostic code) may affect study results 3. Application of information for patient care a. Estimate the post-test probability of a disease, given the pretest probability of the disease and the likelihood ratio for the test b. Calculate absolute risk reduction c. Calculate and interpret the number-needed-to-treat d. Distinguish statistical

significance from clinical importance 4. Using the medical literature a. Given the need for specific clinical information, identify a clear, structured, searchable clinical question b. Identify the study design most likely to yield valid information about the accuracy of a diagnostic test c. Identify the study design most likely to yield valid information about the benefits and/or harms of an intervention d. Identify the study design most likely to yield valid information about the prognosis of a condition D. Principles of Teaching and Learning 1. Educational theory a. Understand the basic principles of adult learning theory (eg, adult learners are self-directed, goal-oriented, practical; need to feel respected, build on life experiences; learn best when learning is based on an existing framework) b. Understand the attributes of an effective learning environment c. Understand the importance of "reflective practice" in teaching and learning d. Identify strategies that motivate

learners e. Recognize the impact of the "hidden curriculum" on learning 2. Feedback and evaluation a. Identify components of effective feedback b. Distinguish between formative and summative feedback c. Distinguish between evaluation and feedback d. Understand the strengths and weaknesses of various methods to evaluate learners 3. Teaching methods a. Understand the strengths and weaknesses of various teaching methods (eg, lecture, small group discussion, bedside teaching, simulation) b. Understand that individuals may learn more effectively with certain teaching methods (eg, reading, hearing, doing) than with others 4. Educational planning a. Understand the role of needs assessment in educational planning b. Distinguish between goals and learning objectives c. Identify components of well-formulated learning objectives d. Recognize the strengths and weaknesses of various educational outcome measures (eg, participant satisfaction, acquisition of knowledge and skills, behavioral

change, patient outcomes) E. Ethics in Research Source: http://www.doksinet 1. 2. 3. 4. 5. Conflicts of interest and commitment a. Evaluate whether an investigator has a conflict of interest during the course of a study b. Understand ways to manage a conflict of interest c. Understand what constitutes a conflict of commitment Professionalism and misconduct in research a. Identify forms of research misconduct (eg, plagiarism, fabrication, falsification) b. Differentiate honest error and differences of opinion from research misconduct c. Understand the criteria for authorship of clinical research publications Principles of research with human subjects a. Understand and apply the three main principles of research ethics articulated in the Belmont Report (eg, respect for persons, beneficence, and justice) b. Understand the role of analysis of risks and benefits in the ethical conduct of research c. Understand the federal regulatory definitions regarding which activities are

considered research d. Understand the federal regulatory definitions regarding when research includes the use of human subjects e. Understand the federal regulatory definition of minimal risk f. Understand the functions of an Institutional Review Board g. Understand the rules that make a study involving children exempt from review by the Institutional Review Board h. Understand the functions of a Data Safety Monitoring Board i. Understand the importance of clinical equipoise in research with human subjects j. Understand the impact of "therapeutic misconception" on clinical research with human subjects k. Understand the ethical considerations of study design (eg, placebo, harm of intervention, deception, flawed design) l. Understand the privacy rules regarding recruitment and participation of subjects in a research study and reporting the results of that study Principles of consent and assent a. Understand what constitutes informed consent in research b. Understand when an

exemption from review by the Institutional Review Board is permissible (eg, medical record review of de-identified data) c. Understand how undue influence can affect obtaining consent for research d. Understand how coercion can affect obtaining consent for research e. Understand the special ethical considerations related to research utilizing children because of their inability to give informed consent f. Distinguish among consent, assent, and permission in research involving children Vulnerable populations a. Recognize that the definition of "children" is related to the underlying clinical intervention in the jurisdiction in which the child is located rather than a fixed nationwide notion of age b. Recognize the types of protections that might be accorded to vulnerable populations (eg, incarcerated individuals, pregnant women, fetuses, children, Source: http://www.doksinet mentally disabled individuals, educationally or economically disadvantaged individuals) c.

Understand the concept of minimal risk as it applies to research involving children d. Understand the circumstances under which research that involves children and that entails greater than minimal risk may be permissible II. Etiology and Pathogenesis A. Musculoskeletal Systems 1. Joints a. Classification 1. Differentiate the types of joints: synarthroses, amphiarthroses, diarthroses 2. Know the locations of the types of joints: synarthroses, amphiarthroses, diarthroses 3. Describe the types of synovial joints according to shape of opposing surfaces of hyaline cartilage: ball and socket, hinge, saddle, plane b. Anatomy 1. Know the embryonic cell type of joints: mesoderm vs ectoderm, endoderm 2. Know the general anatomy of the joint: capsule, synovium, blood vessels, nerves, synovial tissue and fluid, cartilage, bone c. Synovium 1. Know the cellular characteristics of type A synovial membrane cells 2. Know the cellular characteristics of type B synovial membrane cells 3. Identify the

components and functions of synovial fluid 4. Identify the normal concentration of proteins in synovial fluid 5. Understand the mechanics of joint loading and stress distribution to subchondral bone 6. Understand the physiology and pathophysiology of intrasynovial pressure of the knee 7. Know the factors that contribute to the stability of joints d. Articular cartilage 1. Understand the role of type II collagen fibers in articular cartilage 2. Know the matrix components of articular cartilage: collagen, proteoglycans, chondronectin, water, and other materials 3. Know the contribution of the matrix components of articular cartilage vary with age 4. Recognize the cellular composition of articular cartilage and know the function of chondrocytes 5. Identify the physical orientation of chondrocytes in the different zones of articular cartilage 6. Know the homeostasis of chondrocytes and extracellular matrix of cartilage 2. The growing skeleton - Bone a. Understand that serum concentrations

of osteocalcin reflect osteoblastic activity b. Know what affects skeletal bone mass: chronologic age, nutrition, state of general health, heredity, hormones, etc. c. Know the factors affecting bone synthesis: calcium intake, latitude, exposure to sunlight, ingestion of vitamin D, metabolic state, nutritional status, hormones d. Know the effect of exogenous oral corticosteroids on absorption of calcium Source: http://www.doksinet e. f. g. h. i. Know the effects of low levels of calcium intake during adolescence Recognize that inflammation can alter bone metabolism Know the biochemical composition of bone Know the function and origins of osteoblasts, osteocytes, and osteoclasts Know the ratio of cortical/trabecular bone in the one-third distal radius and in the lumbar vertebral bodies j. Know the biochemical assays used to assess bone formation: alkaline phosphatase, osteocalcin, etc. k. Know the biochemical assays used to assess bone resorption: acid phosphatase, urinary calcium to

creatinine ratio, etc. l. Know the clinical measurements and their values and limitations for bone mineral content and bone density: single and dual photon absorptiometry, dual energy x-ray absorptiometry, etc. m. Know the genetic factors that may influence bone mass, such as race, vitamin D receptors, etc. n. Know the physiologic and biochemical events and their regulation that occur during the peak growth period of adolescence: increase in calcitriol concentration, increase in renal reabsorption of phosphate, and increase in gut absorption of calcium and phos o. Know the effects of exercise on bone mass 3. Tendons and ligaments a. Know the major component of tendons: type I collagen b. Know the function of ligaments c. Know the characteristics and locations of entheses in children 4. Muscle a. Know the anatomic characteristics of muscle: fibril, fiber, muscle bundle, sarcomere b. Know the major components of muscle: blood vessels, connective tissue, neurons c. Know the types of

muscle fibers: type I slow/red, type II fast/white d. Know the characteristic ultrastructural appearance of myofilaments: A band, I band, M line, Z band (or disc) e. Know the biochemistry of muscle energy metabolism f. Know the characteristics of the type I and type II muscle fibers: structure and biochemistry 5. Vascular system a. Know the function of fenestrations in synovial microvessels: diffusion-based exchange between plasma and interstitium b. Know the characteristics of the arterial supply in children to the diaphysis and metaphysis of long bones B. Structure of Connective Tissue 1. General a. Understand the general structural relationship of the components of connective tissues 2. Collagens a. Understand the structure and distribution of the various types of collagen b. Recognize the steps in collagen biosynthesis Source: http://www.doksinet c. d. Know the mechanisms that control the production of collagen Know the factors that play a role in collagen degradation 3.

Proteoglycans a. Know the general characteristics of proteoglycans b. Know the role of hyaluronic acid in the organization of extracellular matrix via its interaction with proteoglycans c. Recognize that the serum concentration of keratan sulfate in children reflects cartilage metabolism d. Know the cell types that contain cell-associated proteoglycans e. Know the cellular origin of the proteoglycans in the articular cartilage and the location where aggregation occurs f. Know the effect of growth factors and cytokines on proteoglycan metabolism 4. Other constituents of connective tissue a. Know the properties of elastin b. Know the structures in which elastin is the primary component: ligaments, blood vessels, other tissues c. Know the sources of elastase: pancreas, macrophages, leukocytes, platelets d. Know the effects of aging on elastin fibers e. Know the characteristics of fibronectin f. Understand that laminin is a major constituent of the basement membrane g. Know the properties

of the enzyme that degrades elastin (elastase) C. Inflammation and Immunity 1. Innate Immunity a. General 1. Know the principal components of innate immunity, including physical and chemical barriers: phagocytic cells (neutrophils, macrophages, and natural killer cells): blood proteins (complement, manose-binding lectin, C-reactive protein, coagulation factors): 2. Know that the endoplasmic reticulum is the site where proteins are synthesized 3. Know that the golgi complex is the site where secreted proteins are converted to their final forms and packaged for secretion 4. Know the function of toll-like receptors in innate immunity b. Phagocytes 1. Know that binding of ligands to the 7 transmembrane alpha-helical receptors induces migration of phagocytes through the endothelium as well as production of microbial substances by activation of the respiratory burst 2. Know that phagocytes express the mannose receptor that directly binds microbes for phagocytosis 3. Know that phagocytosis

can be enhanced by coating the microbe with IgG antibodies, C3, and other plasma proteins 4. Know the clinical causes of eosinophilia: parasitism, infection, eosinophilic fasciitis, and allergic diseases 5. Know that 7 transmembrane alpha-helical receptors on neutrophils recognize certain CXC chemokines, such as IL-8 6. Know that macrophages express low levels of MHC class II molecules, which are induced by interferon-gamma Source: http://www.doksinet 7. Understand the role of inducible nitric oxide synthase (iNOS) in inducing free radicals that kill microbes 8. Know that secretion of IL-6 by macrophages induces synthesis of fibrinogen by hepatocytes, which is responsible for the increase in CRP by activating Janus kinases 9. Know that macrophages express Fc receptors, which bind IgG and enhance phagocytosis 10. Know that CD14 expressed on macrophages binds lipopolysaccharide (LPS) from gram-negative bacteria 11. Know that phagocytes kill microbes by production of microbicidal

molecules, such as elastase and free radicals 12. Know that NK cells are a subset of lymphocytes that kill virus- infected cells and secrete interferon-gamma 13. Know that NK cells are predominantly found in the blood and spleen 14. Know that NK cells can be activated by antibody-coated cells, cells infected by viruses, and cells lacking MHC class I molecules 15. Know the function of NK cells, including lysis of virus-infected cells, lysis of tumor cells, and antibody-dependent cell-mediated cytotoxicity 16. Know the phenotypic markers on NK cells, including Fc receptor for IgG (CD 16) 17. Know that mononuclear phagocytes function in innate immunity by phagocytosing microbes and producing cytokines that recruit and activate other inflammatory cells 18. Know that 7 transmembrane alpha-helical receptors recognize short peptides containing N-formyl methionyl residues present only on bacterial proteins 19. Know that 7 transmembrane alpha-helical receptors recognize C5a and a variety of

lipid mediators of inflammation, such as platelet- activating factor, prostaglandin E, and leukotriene B4 c. Blood Proteins 1. Know the association of increased vascular permeability with C3a and C5a 2. Know that the chemotactic ability is a function of C5a 3. Know that C3b is an opsonin 4. Understand the function of C5-9 in cytolysis 5. Understand the classical pathway of complement activation 6. Understand the alternative pathway of complement activation 7. Know the first step in the membrane attack complex: C5b-6-7 8. Know which genes are located on the class III loci of the MHC complex on chromosome 6: C2, Factor B, C4 9. Know the acute phase reactants and their levels in pediatric patients with rheumatic disease 10. Know that stainable iron may be found in macrophages in bone marrow of patients with iron deficiency 11. Know the cellular source of neopterin and that its concentration may be increased in patients with active rheumatic disease 12. Understand the biosynthetic process

and location of complement components Source: http://www.doksinet 13. Understand how complement is regulated to prevent complement activation on host cells 14. Know that leukocytes have receptors for complement components 15. Know how the interaction of the complement, kinin, and clotting pathways facilitates the inflammatory process 16. Know that most acute-phase proteins are synthesized in the liver in response to proinflammatory cytokines 17. Know that C-reactive protein binds to bacterial phospholipids and functions as an opsonin by binding C1q and interacting with phagocyte C1q receptors and by binding directly to Fc gamma-receptors 18. Understand the role of complement receptors 19. Understand the function of the C1 inhibitor 20. Understand the functions of complement, including promotion of phagocytosis of microbes, stimulation of inflammation, and induction of lysis of microbes d. Cytokines 1. General a. Know which cytokines are proinflammatory in the synovium and their

effects b. Know the cytokine profile of Th0, Th1, Th2, and T-regulatory cells c. Identify cytokines produced by monocytes d. Know that proinflammatory cytokines stimulate metalloproteinase activity e. Know that matrix metalloproteinases are involved in the degradation of extracellular matrix 2. TNF a. Know that TNF stimulates endothelial cells and macrophages to secrete chemokines that induce leukocyte chemotaxis and recruitment b. Understand the multistep model of leukocyte recruitment: rolling on endothelium, activation of leukocytes, stable adherence to endo- thelium transmigration through the vessel wall c. Know that, in severe infections, TNF is produced in large amounts and causes systemic and histopathologic abnormalities including fever, production of acute-phase reactants, pyrexia, hypotension, intra- vascular thrombosis, hypoglycemia, and septic shock d. Know that the major cellular source of TNF is activated mononuclear phagocytes, although antigen-stimulated T cells, NK

cells, and mast cells can also secrete TNF e. Know that TNF is structured as a homotrimer f. Know that there are two distinct TNF receptors of molecular size 55 Kd (type 1 TNF receptor) and 75 Kd (type 2 TNF receptor) g. Know that TNF secreted by macrophages stimulates endothelial cells to express e-selectin, ICAM 1 and VCAM 1, which mediate adhesion and migration of leukocytes through the endothelium 3. IL-1 a. Know that IL-1 can have systemic effects, including induction of fever, production of acute-phase reactants, and cachexia Source: http://www.doksinet b. 4. 5. 6. 7. 8. 9. 10. 11. Know that mononuclear phagocytes produce an inhibitor of IL-1, termed IL-1 receptor antagonist c. Know that the principal function of IL-1 is as a mediator of the host inflammatory response to infections and other inflammatory stimuli d. Know that the major cellular source of IL-1 is activated mononuclear phagocytes, but that IL-1 is also produced by neutrophils, epithelial cells, and

endothelial cells e. Know the biologic actions of IL-1, including induction of adhesion molecules on endothelial cells IL-2 a. Know that IL-2 is a growth factor for antigen-stimulated T lymphocytes and is responsible for T-cell clonal expansion after antigen recognition b. Know that IL-2 is produced by T cells c. Know the functions of IL-2, including proliferation of antigen- specific cells, proliferation of NK cells, activation of T cells, and induction of apoptosis in activated T cells IL-4 a. Know the biologic actions of IL-4, including stimulation of B-cell heavy chain class switching to the IgE isotype, development of CD4+ T cells into Th-2 cells, and inhibition of macrophage activation IL-5 a. Know the biologic actions of IL-5, including stimulation of growth and differentiation of eosinophils IL-6 a. Know the role of IL-6 in the pathogenesis of systemic-onset JIA IL-10 a. Know that IL-10 inhibits the expression of co-stimulators and MHC class II molecules on macrophages b. Know

that IL-10 inhibits the production of IL-12 and TNF by activated macrophages IL-12 a. Know that IL-12 stimulates the differentiation of CD4+ T-helper cells into Th1 cells b. Know that IL-12 is a mediator of early innate immune responses to intracellular microbes c. Know that IL-12 stimulates interferon-gamma production by T cells and NK cells d. Know that the principle sources of IL-12 are activated mononuclear phagocytes and dendritic cells e. Know that IL-12 enhances CTL activity Interferon-gamma a. Know the biologic actions of interferon-gamma, including macrophage activation, expression of MHC molecules on APCs, and differentiation of CD4+ helper cells into Th1 cells TGF-beta Source: http://www.doksinet a. Know the biologic actions of TGF-beta, including the inhibition of proliferation and differentiation of T cells and macrophages 12. Chemokines a. Know the biologic actions of chemokines, including recruitment of inflammatory cells to sites of infection and the regulation of

the trafficking of leukocytes through peripheral lymphoid tissues b. Know that chemokines are cytokines that stimulate leukocyte movement and regulate the migration of leukocytes from the blood to the tissues e. Platelets 1. Know that platelets are involved in wound healing and cellular response to injury 2. Know platelet-derived factors with inflammatory properties f. Arachidonic acid 1. Recognize the major arachidonic acid metabolites and how they are biosynthesized: prostaglandins, thromboxanes, leukotrienes 2. Know that arachidonic acid is a polyunsaturated fatty acid contained in the phospholipids of most tissues 3. Understand the role of the cyclooxygenases: catalyze the addition of molecular oxygen 4. Recognize the roles of PGE-2 and PGI-2: mediators of the vascular phase of inflammation 5. Understand that osteoclastic bone resorption is stimulated by PGE-2 and PGI-2: mediators of the vascular phase of inflammation 6. Recognize the sources of leukotrienes: neutrophils,

monocytes, macrophages, mast cells, eosinophils 7. Understand that LTB-4 is a chemoattractant for leukocytes 8. Identify the differences in actions of COX1 and COX2 (cyclooxygenase) 9. Know that the role of lipoxygenases is to catalyze the addition of molecular oxygen 10. Know the mechanisms of action of prostaglandins in the inflammatory process 11. Recognize the sources of prostaglandins 12. Understand the mechanism by which NSAIDs may cause gastric erosions 13. Know the mechanism by which corticosteroids inhibit synthesis of arachidonic acid and its metabolites (prostaglandins and leukotrienes) 14. Know the source and the characteristic features of the vasoactive amines, histamine and serotonin 15. Know that the coenzyme nitric oxide synthetase is responsible for the production of nitric oxide 16. Know the effect of nitric oxide on blood vessels (vasodilation) 2. Adaptive Immunity a. General 1. Know that at the end of an adaptive immune response most of the antigen-stimulated

lymphocytes die by apoptosis 2. Know that CD stands for clusters of differentiation 3. Know that each common variant of a polymorphic gene is called an allele Source: http://www.doksinet 4. Understand the concept of somatic recombination: the creation of functional antigen receptor genes, which occurs only in immature B cells and T cells 5. Identify the intracellular pathways of T- and B-lymphocyte activation 6. Know that the two types of adaptive immunity are humoral immunity and cell-mediated immunity 7. Know that diversity and memory are features of adaptive immunity 8. Understand the clonal selection hypothesis of adaptive immunity: that the antigen receptors of lymphocytes are produced by random DNA recombination events that are not dependent on or influenced by the presence of antigens 9. Know the difference between apoptosis and necrosis 10. Know that hematopoietic stem cells can be identified by expression of CD34 11. Know that the set of genes present on each chromosome is

called a haplotype 12. Know that most T cells recognize only peptides, whereas B cells can specifically recognize peptides, nuclear acids, polysaccharides, lipids, and small chemicals 13. Know that the precursors of lymphocytes arise in the bone marrow 14. Know that the principle cytokine that stimulates the proliferation of B- and T-cell progenitors is IL-7 15. Understand allelic exclusion in B- and T-cell receptors b. Cell-Mediated Immunity 1. MHC a. Know that there are three types of MHC gene products: MHC classes I, II, and III b. Understand the concept of self-MHC restriction, which is a fundamental feature of antigen recognition by T cells c. Know that the physiologic function of MHC molecules is the presentation of peptides to T cells d. Know that the MHC class locus is found on the short arm of chromosome 6 e. Know that MHC class I molecules are constitutively expressed on most nucleated cells f. Know that MHC class II molecules are normally expressed only on dendritic cells, B

cells, macrophages, and a few other cell types g. Know the important candidate susceptibility genes in rheumatic diseases h. Know the role of CD1 i. Know that human MHC molecules are also referred to as human leukocyte antigens (HLA) j. Know that MHC genes are codominantly expressed in each individual k. Know that each MHC molecule has a single peptide-binding cleft that can accommodate many different peptides l. Know that the peptide-binding groove of MHC molecules is composed of a beta-pleated sheet and 2 alpha-helices m. Know that CD4+ T cells are MHC class II restricted and CD8+ T cells are MHC class I restricted n. Know that stable expression of MHC molecules on the cell surface requires the presence of a bound antigenic peptide 2. MHC Class I Source: http://www.doksinet a. Know that the human MHC class I locus is composed of three genes: A, B, and C b. Know that MHC class I is composed of an alpha chain and beta-2 microglobulin c. Know that the polymorphic residues on MHC

class I are found in the alpha-1 and alpha-2 domains d. Know that the alpha-3 domain binds CD8 e. Know that MHC class I accommodates peptide of 8 to 11 residues f. Know that beta-2 microglobulin is invariant among all MHC class I molecules g. Know that increased expression of MHC class I molecules can be induced by interferon-alpha, -beta, and -gamma, and by TNF 3. MHC Class II a. Know that MHC class II is composed of an alpha and a beta chain b. Know that the human MHC class II locus is composed of three genes, DP, DQ, and DR c. Know that the polymorphic residues of MHC class II are found in the alpha-1 and beta-1 domains d. Know that the beta-2 domain binds CD4 e. Know that MHC class II accommodates peptides of 10 to 30 residues or more f. Know that interferon-gamma is the principle cytokine involved in stimulating expression of MHC class II molecules 4. T-Cell Maturation a. Know that T-cell maturation occurs in the thymus b. Understand the concept of positive and negative selection

in T-cell maturation c. Know that thymic cortex contains immature T cells and that thymic medulla contains mature T cells d. Know that cell-mediated immunity is mediated by T lymphocytes 5. Antigen Presentation a. Know the phenotypic markers of T cells, including CD3, CD4, and CD8 b. Know that a subset of immature dendritic cells, Langerhans cells, is present in the epidermis c. Know that professional APCs include dendritic cells, macrophages, and B lymphocytes d. Know the MHC class I and II pathways of antigen processing and presentation, including the role of TAP in the class I pathway and the role of CLIP and HLA-DM in the class II pathway e. Know that T cells recognize and respond to cell-surface-associated antigens but not to soluble antigens f. Know that the antigen receptor for T cells is an alpha-beta or gamma-delta complex g. Know that the specificity of the T-cell receptor is towards the complex formed by peptide and MHC Source: http://www.doksinet h. Know that cells that

display MHC-associated peptides are called antigen-presenting cells (APCs) i. Know that CD4+ and CD8+ T cells preferentially recognize antigen sampled from the extracellular and cytosolic pools, respectively j. Know that rare populations of T cells, such as gamma-delta T cells, can recognize nonpeptide antigens k. Know that dendritic cells are the most effective professional APCs for priming T cells (initiating T-cell-dependent immune responses) l. Understand the role of the proteasome in the generation of peptides for binding to MHC class I 6. T-Cell Activation a. Know that the normal ratio of CD4:CD8 cells in humans is 2:1 b. Know that a small subset of T cells expresses a gamma-delta rather than the alpha-beta T-cell receptor chains c. Know that T costimulation molecules can be delivered through CD28 d. Know the sources of common bacterial superantigens and the mechanism of their activation of T cells e. Know that T-cell proliferation in response to antigen is mediated primarily by

an autocrine growth pathway involving IL-2 and IL-2 receptors f. Know the role of T-cell accessory molecules, including CD8, CD28, CTLA4 (CD152), CD45R, LFA1, L-selectin, and CD44 g. Know the role of each component of the T-cell receptor complex in antigen-mediated T-cell signaling:T-cell receptor-alpha, -beta, -gamma, and -delta chains; CD3-gamma, -delta, -epsilon, and -zeta chains h. Know that the structure of the alpha-beta T-cell receptor is a heterodimer consisting of 2 transmembrane polypeptide chains covalently linked by disulfide bonds i. Know that the sequence variability in T-cell receptors is concentrated in the third complementary-determining region (CDR3) j. Know that the affinity of a T-cell receptor for peptide MHC complexes is lower than that of most antibodies k. Know that CD3 is invariant within a species l. Know that the majority of gamma-delta T cells do not express CD4 or CD8 m. Know that CD4 and CD8 bind to nonpolymorphic regions of the MHC molecules and transduce

signals that help initiate T-cell activation n. Know that T cells require two distinct extracellular signals in order to initiate proliferation and differentiation into effector cells o. Know that CD28 binds B7.1 and B72 on professional APCs, resulting in delivery of a second signal to the T cell p. Know that CTLA4 binds B7.1 and B72, resulting in inhibition of T-cell activation by countering signals delivered by CD28 q. Know that T-cell adhesion molecules include integrins, selectins, and CD44 r. Know that the major functions of T-cell integrins are to mediate adhesion to APCs, endothelial cells, and extracellular matrix proteins s. Know that macrophages and B cells are more efficient activators of effector T cells than of naive T cells Source: http://www.doksinet t. Know that the high affinity IL-2 receptor is present in low amounts on naive T cells and memory T cells but in high amounts on activated T cells u. Know that the principle cytokine produced by naive T cells is IL-2 v.

Understand that the allelic exclusion of T cells applies only to the beta chain, whereas there is no allelic exclusion in the alpha chain, and that up to 30% of mature peripheral T cells express two different T-cell receptors with different alpha chains b 7. T-Cell Effector Function a. Know the principal functions of Th1 cells, including phagocyte- mediated defense against infections with intracellular microbes by production of interferon-gamma b. Know the function of cytotoxic lymphocytes, including lysis of virus- infected cells, tumor cells, and allografts c. Know the function of Th2 cells, including IgE- and eosinophil/mast cell-mediated immune reactions by secretion of IL-4, IL-5, and IL-13 d. Know that the principal mechanism of CTL-mediated cytosis is the delivery of cytotoxic granule proteins, such as perforin and granzymes, to the target cell e. Know that antigen-activated CD4+ helper T cells activate B lymphocytes and macrophages by secreting cytokines and by expressing CD40

ligand, which engages CD40 on these cells f. Know that naive T cells express the CD45 RA isoform and that memory T cells express the CD45 RO isoform g. Know that lymphocytes that infiltrate the synovium express the CD45 RO isoform h. Understand the role of adjuvants in T-cell activation i. Know that the responses of CD4+ T cells are initiated in the peripheral lymphoid organs to which protein antigens are transported after being collected from their portal of entry j. Know that in the effector phase of CD4+ T-cell responses, previously activated effector or memory cells may recognize and respond to antigens in nonlymphoid tissues k. Know that activated macrophages are the effector cells of cell- mediated immunity and that they eliminate microbes and other sources of antigen l. Know that cell killing by CD8+ cytotoxic lymphocytes is antigen specific and contact dependent c. Humoral Immunity 1. General a. Know that humoral immunity is mediated by antibodies produced by B lymphocytes b.

Know that humoral immunity is a principal defense against extra- cellular microbes and their toxins c. Understand the difference between active and passive immunity 2. Antibody a. Know the structure of antibodies Source: http://www.doksinet b. Know that all immunoglobulin heavy chain genes are found on human chromosome 14 c. Know the idiotypic characteristics of immunoglobulins d. Know the members and the function of the Ig supergene family (Ig, TCR, HLA, LFA, etc) e. Know that the surface antigen receptor for B lymphocytes is immunoglobulin f. Know how monoclonal antibodies are derived g. Know that antigen binding by antibody molecules is primarily a function of the hypervariable regions of VH and VL h. Know that IgG and IgE circulate as monomers, whereas secreted forms of IgA and IgM circulate as dimers and pentamers, respectively i. Know that IgA is important in mucosal immunity and neonatal passive immunity j. Know that IgG functions in opsonization, complement activation,

antibody-dependent cell-mediated cytotoxicity, neonatal immunity, and passive immunity k. Know that IgM functions in complement activation l. Know that IgE mediates immediate hypersensitivity 3. B-Cell Maturation a. Know that mature B cells co-express IgM and IgD b. Know that B-cell maturation occurs in the bone marrow c. Know the stages of B-cell development: stem cell, pro-B cell, pre-B cell, immature B cell, and mature B cell d. Know that immature B lymphocytes express membrane IgM e. Know that activated and memory B cells express IgG, IgA, or IgE 4. Antigen Presentation a. Know that antibody responses to protein antigens are T-cell dependent, while antibody responses to nonprotein antigens, such as poly- saccharides and lipids, are T-cell independent b. Understand the interactions of B cells and T cells in the B-cell response to protein antigens c. Know that the simultaneous engagement of B-cell Ig and Fc-gamma receptors by antigen-antibody complexes inhibits B-cell activation 5.

B-Cell Activation a. Know the phenotypic markers for B lymphocytes, including Fc receptors, MHC class II, CD19, and CD21 b. Know how isotype switching occurs in activated B cells c. Understand the concepts of somatic mutation and affinity maturation in B cells d. Know that the activation of B cells requires, in addition to antigen, second signals that may be provided by complement proteins that bind to complement receptors on B cells e. Know that antigen binding to membrane Ig enhances the expression of costimulators such as B7.1 and B72 that increase the ability of the B lymphocyte to activate helper T cells Source: http://www.doksinet 6. B-Cell Effector Function a. Know that the antigen-presenting function of B cells is essential for helper T-cell-dependent antibody production b. Understand the differences between primary and secondary antibody responses following antigen exposure c. Know the roles of cytokines in antibody responses, including augmentation of B-cell proliferation

and differentiation and promotion of antibody isotype switching d. Know that Fc-epsilon receptors are found on mast cells, basophils, and eosinophils e. Know that Fc-gamma receptor 3 (CD16) is found on NK cells and is responsible for antibody-dependent cell-mediated cytotoxicity d. Lymph Nodes 1. Know that primary follicles contain mostly mature naive B lymphocytes 2. Know that B cells reside in the lymphoid follicles and T cells reside in the paracortex 3. Know that germinal centers develop in response to antigenic stimulation and contain proliferating B cells 4. Know that lymphocytic extravasation from the blood into peripheral lymph nodes occurs at the high endothelial venules 5. Know that dendritic cells are derived from mononuclear phagocytes 6. Know that dendritic cells are accessory cells that play important roles in the induction of T-lymphocyte responses to antigens 7. Know that the main function of dendritic cells is to capture and transport protein antigens to draining lymph

nodes 8. Know that mature dendritic cells reside in T-cell-containing regions of lymph nodes where they display antigens to T cells e. Spleen 1. Know that the spleen is the major site of immune responses to bloodborne pathogens 2. Know that splenic T cells reside primarily in the periarteriolar lymphoid sheaths 3. Know that splenic B cells reside primarily in the follicles and germinal centers f. Immunologic Tolerance 1. Know the mechanisms of central and peripheral tolerance 2. Understand the role of Fas and Fas ligand in activation-mediated T-cell apoptosis 3. Understand the concept of oral tolerance 3. Immunity to Microbes a. Know that the principal mechanisms of innate immunity to extra- cellular bacteria are complement activation, phagocytosis, and the inflammatory response b. Know that the principal adaptive immune response to extracellular bacteria is humoral immunity c. Know that the major protective immune response against intracellular bacteria is cell-mediated immunity by NK

cells d. Know that the principal mediators of immunity to fungi are neutrophils and macrophages Source: http://www.doksinet e. Know that immunity to viruses is mediated by NK cells, cytotoxic lymphocytes, and antibodies f. Know that the principal defense against helminthic infections is Th2 cells, which induce production of IgE antibodies and activation of eosinophils 4. Diseases Caused by Immune Responses a. Hypersensitivity 1. Type 1: Immediate Hypersensitivity a. Know that mast cells and basophils are activated by crosslinking of Fc-epsilon receptor-1 molecules, which occurs by binding of multi- valent antigens to the attached IgE molecules b. Know that activated mast cells rapidly release histamine and neutral serine proteases, including tryptase and chymase c. Know that mast-cell activation and basophil activation result in synthesis and release of lipid-derived mediators, such as prostaglandin D2, leukotrienes, and platelet-activating factor, that effect bronchial smooth

muscle, blood vessels, and leukocytes d. Understand the sequence of events in immediate hypersensitivity reactions e. Understand the cause of the wheel and flare reaction to allergens f. Understand the role of IgE in immediate hypersensitivity g. Know that atopic individuals produce high levels of IgE in response to particular antigens and that normal individuals synthesize other Ig isotypes and only a small amount of IgE h. Know that IL-4 promotes eosinophil recruitment i. Know that repeated exposure to a particular antigen is necessary to develop an allergic reaction to that antigen j. Know that mast cells, basophils, and eosinophils are the effector cells of immediate hypersensitivity reactions and allergic disease k. Know that mast cells and basophils produce many different cytokines that may contribute to allergic inflammation, including TNF, IL-1, IL-4, IL-5, IL-6, IL-13 l. Know that anaphylactic shock is a consequence of release by mast cells and basophils of mediators that

cause vasodilation and exudation of plasma m. Know that a principal protective function of IgE-initiated immune responses is the eradication of parasites n. Know that mast cells play an important protective role as part of the innate immune response to bacterial infections 2. Type 2:Antibody-Mediated Hypersensitivity a. Know that autoimmune hemolytic anemia is caused by antibodies to erythrocyte membrane proteins b. Know the use and limitations of laboratory tests used to detect immune complexes: Raji cell assay, Clq binding assay c. Know that pemphigus vulgaris is caused by antibodies to desmoglein 1 and 3 and E-cadherin 3. Type 3: Immune Complex-Mediated Hypersensitivity a. Know that immune complex-mediated hypersensitivity is the cause of lupus nephritis, polyarteritis nodosa, and post-streptococcal glomerulonephritis Source: http://www.doksinet 4. b. c. d. e. f. Type 4:T-Cell-Mediated (Delayed-Type) Hypersensitivity a. Know that T-cell-mediated hypersensitivity is involved

in type 1 diabetes and rheumatoid arthritis b. Understand the concept of molecular mimicry as a hypothesis for autoimmune disease c. Understand the role of macrophages in DTH B-Cell Immunodeficiencies 1. Know that X-linked agammaglobulinemia (Bruton) is associated with a decrease in oral serum Ig isotypes and reduced numbers of B cells 2. Know that in X-linked agammaglobulinemia the maturation number and functions of T cells are generally normal T-Cell Immunodeficiencies 1. Know that patients with DiGeorge syndrome have decreased count of T cells, normal count of B cells, and normal or decreased serum Ig concentration 2. Know that DiGeorge syndrome is caused by anomalous development of third and fourth branchial pouches leading to thymic hypoplasia 3. Know that in patients with DiGeorge syndrome T-cell function tends to improve with age and is often normal by 5 years of age 4. Know that approximately 50% of severe combined immunodeficiencies are X-linked and due to mutations in the

gene for the common gamma chain for cytokines 5. Know that adenosine deaminase-deficient SCID patients have decreased counts of both T cells and B cells Defects in Lymphocyte Activation 1. Understand the broad clinical spectrum of serum IgA deficiency 2. Know the most common genetic cause of X-linked hyper-IgM immunodeficiency 3. Know that the clinical features of X-linked hyper-IgM syndrome are caused by deficient T-cell dependent, B-cell, and macrophage activation 4. Know the clinical features of common variable immunodeficiency 5. Know the clinical features of X-linked lymphoproliferative disease 6. Know the clinical features of bare lymphocyte syndrome 7. Know that a congenital deficiency in the tap genes leads to lack of MHC class I expression and susceptibility to respiratory tract bacterial infections 8. Know that 1 in 500 healthy blood donors has isolated IgA deficiency Immunodeficiency Associated with Other Inherited Diseases 1. Know the clinical features of the

Wiskott-Aldrich syndrome 2. Know the clinical features of ataxia telangiectasia Diseases of Innate Immunity 1. Know the clinical and histopathologic characteristics of Chediak- Higashi syndrome 2. Know the clinical features of chronic granulomatous disease 3. Know that chronic granulomatous disease is caused by mutations in a component of the phagocyte oxidase enzyme 4. Know the clinical features of leukocyte adhesion deficiencies Source: http://www.doksinet 5. Know that LAD-1 is caused by mutations in the beta chain (CD18) of beta-2 integrins g. Acquired Immunodeficiencies 1. Know the clinical features of acquired immunodeficiency syndrome (AIDS) 2. Know that HIV is a member of the lentivirus family of retroviruses 3. Know the cell surface receptors that are bound by HIV (CD4 and various chemokine receptors) 5. Laboratory Techniques Commonly Used in Immunology a. Understand the technique of Southern blot hybridization b. Understand how enzyme-linked immunosorbent assays (ELISAs)

are performed c. Understand the technique of immunoprecipitation d. Understand the technique of Western blotting e. Understand the technique of flow cytometry f. Understand the techniques of immunofluorescence and immunohistochemistry g. Understand the technique of Western blot hybridization h. Understand the technique of DNA microarrays i. Understand the technique of polymerase chain reaction (PCR) j. Understand how transgenic mice are generated k. Understand how gene knockout mice are generated D. Animal Models 1. Models of chronic arthritis a. Understand the rodent models of adjuvant-induced arthritis and their relevance to human disease b. Recognize that specific epitopes displayed by type II collagen may elicit T-cell reactivity in patients with arthritis c. Understand the rodent models of collagen-induced arthritis and their relevance to human disease d. Understand the rodent models of streptococcal cell wall-induced arthritis 2. Models of SLE a. Know the strengths and weaknesses

and pathomechanisms of the different models of SLE: New Zealand mice, MRL/lpr mice, BXSB mice, Aleutian disease in mink b. Know that the g/d mouse has a defect in the Fas ligand and in the process of apoptosis c. Know that the lpr mouse has a defect in the Fas gene and in the process of apoptosis 3. Models of Scleroderma a. Be familiar with the models of scleroderma: tight mouse, tight-skinned chicken b. Know that experimental chronic graft-versus-host disease is a model of scleroderma 4. Models of Myositis a. Know that murine polymyositis-like disease is associated with infection with viruses such as the coxsackievirus 5. Models of Spondyloarthropathy a. Know that the B27 transgenic rat spontaneously develops a spondyloarthropathy-like disease III. Drug Therapy Source: http://www.doksinet A. Basic Pharmacokinetics 1. Absorption a. Understand that generic drugs may vary by up to 20% in bioavailability according to FDA regulations on bioequivalence b. Understand the variability of

solid versus liquid bioavailability of drugs c. Know that enantiomers may differ in absorption 2. Protein binding a. Understand that drug-protein binding influences metabolism b. Understand the influence of relative protein binding on drug interactions c. Understand that drug-protein binding influences distribution 3. Drug distribution a. Understand variability in distribution of drugs based on intravascular/extracellular/intracellular compartments b. Understand the importance of the blood-brain barrier in determining drug distribution and toxicity c. Understand the variability in the distribution of drugs due to fat solubility 4. Metabolism a. Differentiate first-order (linear) kinetics from zero-order (saturation) kinetics b. Understand the relationships between half-life and dosing intervals c. Understand what factors influence the T 1/2 of a drug 5. Elimination a. Understand the factors that influence elimination of a drug b. Recognize which commonly used drugs for rheumatic

diseases are expressed in breast milk 6. Compliance a. Recognize that noncompliance with drug therapy is a major factor in variability of clinical response B. Nonsteroidal Anti-inflammatory Drugs (NSAIDs) 1. Indications and contraindications a. Understand the indications for the use of NSAIDs b. Understand the contraindications for the use of NSAIDs 2. Effectiveness in rheumatic diseases a. Know the conclusions of various studies on the use of NSAIDSs in children with arthritis 3. Pharmacology, general a. Understand the mechanism of action of NSAIDs (interference with cyclooxygenase 1 and 2) b. Know the physiologic effects of arachidonic acid metabolites on various organs c. Understand the mechanism of clearance of NSAIDs d. Understand the time course of analgesic and anti-inflammatory actions of NSAIDs e. Understand the range of variability of individual responses to specific NSAIDs 4. Pharmacology, drug-specific a. Know the FDA-approved NSAIDs for pediatric use b. Know the pediatric

doses for the commonly used NSAIDs c. Recognize the NSAIDs available in liquid form d. Understand the pharmacokinetics of NSAIDs Source: http://www.doksinet e. Understand the biologic activity of NSAIDs 5. Side effects/toxicities a. Understand the effects of acetylsalicylate on platelets b. Recognize salicylate toxicity c. Recognize the mucocutaneous adverse reactions of NSAIDs d. Understand the mechanism of NSAID-induced gastropathy e. Understand the effects of NSAIDs on renal blood flow f. Recognize the various types of NSAID-induced renal toxicity and their mechanisms (eg, interstitial nephritis) g. Recognize the various types of hypersensitivity reactions to NSAIDs h. Understand the concern about Reye syndrome and salicylates i. Understand the different toxicity profiles of inhibitors of cyclooxygenase 1 and 2 j. Understand methods to prevent or decrease gastrointestinal side effects of NSAIDs k. Recognize the various forms of hepatotoxicity from NSAIDs l. Recognize the

diversity of central nervous system adverse effects of NSAIDs m. Understand the effects of NSAIDs on coagulation n. Plan laboratory monitoring for NSAID toxicity o. Plan the management of various types of NSAID toxicity (eg, interstitial nephritis) p. Plan treatment of salicylate toxicity q. Recognize papillary necrosis as an NSAID side effect r. Plan the management of papillary necrosis due to NSAID therapy s. Recognize the relationship between nasal polyps and bronchospasm with NSAIDS 6. Drug interactions a. Understand significant interactions of NSAIDs with other drugs (eg, methotrexate, warfarin) 7. Pregnancy and lactation a. Understand the effect of NSAIDs on pregnant women, the fetus, and breast-feeding the neonate C. Hydroxychloroquine 1. Indications and contraindications a. Understand the indications for the use of antimalarial drugs in childhood rheumatic diseases b. Recognize the contraindications for the use of antimalarial drugs in childhood rheumatic diseases 2.

Effectiveness in rheumatic disease a. Recognize the efficacy studies of hydroxychloroquine in JRA b. Recognize the efficacy studies of hydroxychloroquine in SLE c. Recognize the efficacy studies of hydroxychloroquine in discoid lupus 3. Pharmacology a. Understand the pharmacokinetics of hydroxychloroquine b. Understand the biologic activity of hydroxychloroquine 4. Side effects/toxicities a. Recognize the adverse reactions to hydroxychloroquine in children b. Recognize the three types of ocular toxicity associated with antimalarial drugs c. Understand how to test for ocular toxicity associated with antimalarial drugs d. Understand the reversibility of ocular toxicity of hydroxychloroquine Source: http://www.doksinet e. f. Know the relationship between dosage of antimalarial drugs and ocular toxicity Be aware of the effects on serum lipoprotein concentration of hydroxychloroquine 5. Drug interactions a. Recognize significant interactions between hydroxychloroquine and other drugs 6.

Pregnancy and lactation a. Understand the effect of hydroxychloroquine on pregnant women, the fetus, and breast-feeding the neonate D. Sulfasalazine 1. Indications and contraindications a. Understand the indications for the use of sulfasalazine in childhood rheumatic diseases b. Recognize the contraindications for the use of sulfasalazine in childhood rheumatic diseases 2. Effectiveness in rheumatic disease a. Know the efficacy studies of sulfasalazine in children with arthritis b. Know the efficacy studies of sulfasalazine in spondyloarthropy c. Know the efficacy studies of sulfasalazine in arthritis of inflammatory bowel disease 3. Pharmacology a. Understand the pharmacokinetics of sulfasalazine b. Understand the biologic activity of sulfasalazine 4. Side effects/toxicities a. Recognize the hematologic adverse reactions to sulfasalazine in children b. Recognize the cutaneous adverse reactions of sulfasalazine c. Recognize side effects, other than hematologic and cutaneous, associated

with sulfasalazine use d. Plan treatment of Stevens-Johnson syndrome secondary to sulfasalazine 5. Drug interactions a. Recognize significant interactions between sulfasalazine and other drugs 6. Pregnancy and lactation a. Understand the effect of sulfasalazine on pregnant women and the fetus E. Colchicine 1. Indications and contraindications a. Understand the indications for the use of colchicine in childhood rheumatic diseases b. Recognize the contraindications for the use of colchicine in childhood rheumatic diseases 2. Effectiveness in rheumatic disease a. Recognize the efficacy studies of colchicine in rheumatic diseases 3. Pharmacology a. Understand the biologic activity of colchicine b. Understand the pharmacokinetics of colchicine 4. Side effects/toxicities a. Recognize acute colchicine toxicity b. Recognize chronic colchicine toxicity c. Plan management of colchicine toxicity Source: http://www.doksinet d. Plan monitoring for colchicine toxicity 5. Drug interactions a.

Recognize significant interactions between colchicine and other drugs 6. Pregnancy and lactation a. Understand the effect of colchicine on pregnant women and the fetus F. Glucocorticoid Drugs 1. Indications and contraindications a. Understand the indications for the use of glucocorticoids in pediatric rheumatic diseases b. Understand the contraindications for the use of glucocorticoids in pediatric rheumatic diseases c. Understand the indications for pulse intravenous glucocorticoids in pediatric rheumatic diseases d. Understand the contraindications for pulse intravenous glucocorticoids in pediatric rheumatic diseases 2. Effectiveness in rheumatic disease a. Understand the efficacy studies of glucocorticoids in children with chronic arthritis b. Understand the efficacy studies of glucocorticoids in discoid lupus c. Understand the efficacy studies of glucocorticoids in spondyloarthropathy 3. Pharmacology a. Understand the pharmacokinetics of oral glucocorticoids b. Understand the

biologic activity of glucocorticoids c. Understand the pharmacokinetics of high-dose (mega-dose) oral glucocorticoids d. Understand the pharmacokinetics of intravenous glucocorticoids e. Understand the pharmacokinetics of glucocorticoids administered every other day 4. Side effects/toxicities a. Understand the mechanism by which glucocorticoids produce osteoporosis b. Understand the mechanism by which glucocorticoids produce growth suppression c. Plan management of vertebral compression fractures in patients receiving glucocorticoids d. Recognize and plan the management of avascular necrosis secondary to glucocorticoid therapy e. Understand the effects of glucocorticoid therapy in masking clinical features of infections f. Recognize opportunistic infections in patients receiving glucocorticoids g. Recognize and manage acute and chronic adrenal insufficiency caused by glucocorticoid therapy h. Plan the management of children receiving glucocorticoid therapy undergoing major physiologic

stress (surgery, trauma, sepsis) i. Understand the effect of high-dose glucocorticoid therapy on the menstrual cycle j. Recognize and plan the management of diabetes secondary to glucocorticoid treatment k. Recognize and plan the management of hypertension secondary to glucocorticoid treatment l. Recognize and plan the management of cholesterol and lipid abnormalities secondary to glucocorticoid treatment Source: http://www.doksinet m. Recognize and plan the management of obesity secondary to glucocorticoid treatment n. Recognize and plan the management of hirsutism secondary to glucocorticoid treatment o. Recognize and plan the management of myopathy secondary to glucocorticoid treatment p. Recognize and plan the management of cataracts secondary to glucocorticoid treatment q. Know the different preparations of glucocorticoids available for use in children r. Recognize and plan the management of established glucocorticoid- induced osteoporosis s. Plan the management that will

prevent glucocorticoid-induced osteoporosis 5. Drug interactions a. Recognize significant interactions between glucocorticoid drugs and other drugs 6. Intravenous pulse therapy a. Plan intravenous pulse glucocorticoid therapy in children with rheumatic diseases b. Recognize the results of efficacy studies of intravenous pulse therapy in children with rheumatic diseases c. Recognize and plan management of the side effects of pulse intravenous glucocorticoid therapy 7. Intra-articular glucocorticoid treatment a. Understand the indications for the use of intra-articular glucocorticoids, including knowledge of specific preparations b. Recognize the contraindications for the use of intra-articular glucocorticoids c. Recognize the side effects of intra-articular glucocorticoid treatment d. Recognize the results of effectiveness studies of intra-articular glucocorticoids in childhood arthritis 8. Pregnancy and lactation a. Understand the effect of glucocorticoid therapy on pregnant women, the

fetus, and in breast-feeding neonates b. Know the glucocorticoids that have biologic activity in the fetus G. Methotrexate 1. Indications and contraindications a. Understand the indications for the use of methotrexate in the treatment of childhood rheumatic diseases b. Understand the contraindications for the use of methotrexate in the treatment of childhood rheumatic diseases 2. Pharmacology a. Understand the pharmacokinetics of methotrexate b. Understand the biologic activity of methotrexate 3. Side effects a. Plan management of side effects of methotrexate b. Recognize the available studies of the safety of methotrexate in childhood rheumatic diseases c. Understand the indications for liver biopsy in children receiving methotrexate therapy Source: http://www.doksinet d. e. f. Plan laboratory monitoring of side effects of methotrexate Understand the rationale for the use of weekly methotrexate therapy Understand the relationship between blood concentrations, time, and toxicity of

methotrexate g. Understand the gastrointestinal side effects of methotrexate h. Understand the hepatic side effects of methotrexate i. Understand the mucosal side effects of methotrexate j. Understand the pulmonary side effects of methotrexate k. Understand the hematologic side effects of methotrexate l. Understand the risks of infection with methotrexate therapy m. Understand the relationship of side effects of concurrent use of methotrexate and NSAIDs n. Be aware of the use of folic acid and folinic acid in the management of methotrexate side effects and toxicity o. Know the data about the risk of malignancy in patients with rheumatoid arthritis treated with methotrexate p. Understand the long-term effect on gonadal function of methotrexate 4. Drug interactions a. Understand the interaction between methotrexate and NSAIDs b. Understand the usefulness of combination therapy with methotrexate and other long-acting immunomodulatory or immunosuppressive therapies c. Understand the

potential adverse drug interactions with combination therapy with methotrexate and other long-acting immunomodulatory or immunosuppressive therapies d. Understand the potential interaction between methotrexate and trimethoprim 5. Pregnancy and lactation a. Understand the studies of teratogenicity of methotrexate b. Plan management of sexually active females receiving methotrexate therapy H. Azathioprine 1. Indications and contraindications a. Understand the indications for the use of azathioprine in the treatment of childhood rheumatic diseases b. Understand the contraindications for the use of azathioprine in the treatment of childhood rheumatic diseases 2. Pharmacology a. Understand the pharmacology, metabolism, and elimination of azathioprine b. Understand the biologic activity of azathioprine 3. Side effects a. Recognize common side effects of azathioprine therapy b. Understand the available studies of the safety of azathioprine in childhood rheumatic diseases c. Understand the

hematologic side effects of azathioprine d. Understand the idiopathic adverse response to azathioprine e. Understand the risk of infection with azathioprine therapy f. Plan laboratory monitoring of side effects of azathioprine in children with and without thiopurine methyltransferase deficiency Source: http://www.doksinet g. h. Plan management of side effects of azathioprine Understand studies of oncogenicity in patients with rheumatic diseases treated with azathioprine i. Understand the long-term effect on gonadal function of azathioprine in patients with rheumatic disease 4. Drug interactions a. Understand the significant interactions between azathioprine and other medications b. Understand the usefulness of combination therapy with azathioprine and other long-acting immunomodulatory or immunosuppressive therapies c. Understand the potential adverse drug interactions with combination therapy with azathioprine and other long-acting immunomodulatory or immunosuppressive therapies

5. Pregnancy and lactation a. Understand the effects of azathioprine therapy on pregnant women and the fetus b. Plan management of sexually active females receiving azathioprine therapy I. Leflunomide 1. Be aware of the use of leflunomide in patients with childhood rheumatic diseases, including toxicity, surveillance, and precautions in childbearing age females 2. Understand the efficacy studies of leflunomide in rheumatic disease 3. Understand the pharmacokinetics of leflunomide 4. Understand the elimination of leflunomide 5. Recognize the side effects of leflunomide J. Cyclophosphamide 1. Indications and contraindications a. Understand the indications for the use of oral daily cyclophosphamide in the treatment of childhood rheumatic diseases b. Understand the contraindications for the use of oral daily cyclophosphamide in the treatment of childhood rheumatic diseases c. Understand the indications for the use of intravenous pulse cyclophosphamide in the treatment of childhood

rheumatic diseases d. Understand the contraindications for the use of intravenous pulse cyclophosphamide in the treatment of childhood rheumatic diseases 2. Pharmacology a. Understand the pharmacokinetics of cyclophosphamide b. Understand the biologic activity of cyclophosphamide 3. Side effects a. Understand methods to decrease cyclophosphamide toxicity b. Plan management of side effects of cyclophosphamide therapy c. Recognize the available studies of the safety of cyclophosphamide therapy in childhood rheumatic diseases d. Understand the differences between side effects of cyclophosphamide administered intravenously and orally e. Understand the hematologic side effects of cyclophosphamide therapy f. Understand bladder toxicity in cyclophosphamide therapy g. Understand the risk of infection with cyclophosphamide therapy h. Plan laboratory monitoring of side effects of cyclophosphamide therapy Source: http://www.doksinet i. Understand studies of oncogenicity in patients with

rheumatic diseases treated with cyclophosphamide j. Understand the relationship between hemorrhagic cystitis and bladder carcinoma in patients treated with cyclophosphamide k. Understand the long-term effect on gonadal function of cyclophosphamide in patients with rheumatic disease l. Be aware of strategies that may minimize gonadal toxicity of cyclophosphamide therapy 4. Drug interactions a. Understand the significant interactions between cyclophosphamide and other medications b. Understand the usefulness of combination therapy with cyclophosphamide and other long-acting immunomodulatory or immunosuppressive therapies c. Understand the potential adverse drug interactions with combination therapy with cyclophosphamide and other long-acting immunomodulatory or immunosuppressive therapies 5. Pregnancy and lactation a. Understand the effects of cyclophosphamide therapy on pregnant women and the fetus b. Plan management of sexually active females receiving cyclophosphamide therapy K.

Cyclosporin A/Calcineurin inhibitors 1. Indications and contraindications a. Understand the indications for the use of cyclosporin A in childhood rheumatic diseases b. Recognize the contraindications for the use of cyclosporin A in childhood rheumatic diseases 2. Effectiveness in rheumatic disease a. Understand the efficacy studies of cyclosporin A in rheumatoid arthritis and other rheumatic diseases 3. Pharmacology a. Understand the pharmacokinetics of cyclosporin A b. Understand the biologic activity of cyclosporin A 4. Side effects a. Recognize the available studies of the safety of cyclosporin A in childhood rheumatic diseases b. Recognize common cyclosporin A side effects c. Understand the risk of infection with cyclosporin A therapy d. Understand renal toxicities of cyclosporin A therapy e. Understand the neurologic toxicities of cyclosporin A therapy f. Understand the risk of lymphoproliferative disorders in patients with rheumatic diseases treated with cyclosporin A g. Plan

monitoring for cyclosporin A toxicity h. Plan management of cyclosporin A toxicity i. Understand the risk of hypomagnesemia with cyclosporin A therapy j. Understand the risk of hypophosphatemia with cyclosporin A therapy k. Plan management of hypomagnesemia with cyclosporin A therapy Source: http://www.doksinet l. Plan management of hypophosphatemia with cyclosporin A therapy 5. Drug interactions a. Recognize significant interactions between cyclosporin A and macrolide antibiotics b. Recognize significant interactions between cyclosporin A and other drugs c. Know the factors that influence serum concentrations of cyclosporin A (eg, grapefruit juice) 6. Pregnancy and lactation a. Understand the effect of cyclosporin A on pregnant women and the fetus L. Biologic Agents 1. Indications and contraindications a. Recognize the biologic agents used in the treatment of rheumatic diseases (eg, etanercept, infliximab, anakinra, abatacept, adalimumab, rituximab, tocilizumab, MRA) b. Understand

the limitations in the use of biologic agents for the treatment of pediatric rheumatic diseases c. Understand the indications for the use of therapies directed at modulation of cell surface receptors in the treatment of rheumatic diseases d. Understand the contraindications for the use of therapies directed at modulation of cell surface receptors in the treatment of rheumatic diseases e. Understand the indications for the use of therapies directed at modulation of cytokines in childhood rheumatic diseases f. Understand the contraindications for the use of therapies directed at modulation of cytokines in childhood rheumatic diseases 2. Effectiveness in rheumatic disease a. Understand the efficacy studies of biologic agents in pediatric rheumatic disease b. Know the efficacy of tumor necrosis factor inhibitors in rheumatic diseases c. Know the pediatric prescribing options of the biologic agents for different age groups 3. Pharmacology a. Understand the proposed mechanisms of action of

cell surface receptor modulation b. Understand the pharmacokinetics of various biologic agents used to treat rheumatic diseases c. Understand the biologic activity of various biologic agents used to treat rheumatic diseases d. Know the basic principles of structural or pharmacologic design of biologic agents for the treatment of children with rheumatic diseases 4. Side effects a. Recognize the available studies of the safety of biologic agents in childhood rheumatic diseases b. Recognize common side effects of biologic agents c. Understand the risk of infection with therapy with biologic agents d. Plan monitoring for toxicity of biologic agents e. Plan management of toxicity of biologic agents f. Know the side effects of available biologic agents g. Recognize the differences between the TNF drugs 5. Drug interactions Source: http://www.doksinet a. Recognize significant interactions between biologic agents and other drugs 6. Pregnancy and lactation a. Understand the effect of

biologic agents on pregnant women and the fetus M. Anti-TNF-alpha agents 1. Indications and contraindications a. Understand the indications for the use of anti-TNF-alpha agents in the treatment of childhood rheumatic diseases b. Understand the contraindications for the use of anti-TNF-alpha agents in the treatment of childhood rheumatic diseases 2. Pharmacology a. Understand the pharmacology of anti-TNF-alpha agents b. Understand the biologic activity of anti-TNF-alpha agents c. Understand the differences between the various anti-TNF-alpha agents d. Prescribe the appropriate dose of the various anti-TNF-alpha agents 3. Side effects a. Recognize common side effects of anti-TNF-alpha agent therapy b. Understand the available studies of the safety of anti-TNF-alpha agents in childhood rheumatic diseases c. Understand the hematologic side effects of anti-TNF-alpha agents d. Understand the idiopathic adverse response to anti-TNF-alpha agents e. Understand the risk of infection with

anti-TNF-alpha agent therapy f. Plan laboratory monitoring of side effects of anti-TNF-alpha agents g. Plan management of side effects of anti-TNF-alpha agents h. Understand studies of oncogenicity in patients with rheumatic diseases treated with anti-TNF-alpha agents 4. Drug interactions a. Understand the significant interactions between anti-TNF-alpha agents and other medications b. Understand the usefulness of combination therapy with anti-TNF-alpha agents and other long-acting immunomodulatory or immunosuppressive therapies c. Understand the potential adverse drug interactions with combination therapy with anti-TNF-alpha agents and other long-acting immunomodulatory or immunosuppressive therapies 5. Pregnancy and lactation a. Understand the effects of anti-TNF-alpha agent therapy on pregnant women, the fetus, and breast-feeding the newborn N. Anakinra 1. Indications and contraindications a. Understand the indications for the use of anakinra in the treatment of childhood rheumatic

diseases b. Understand the contraindications for the use of anakinra in the treatment of childhood rheumatic diseases 2. Pharmacology a. Understand the pharmacology of anakinra b. Understand the biologic activity of anakinra c. Prescribe the appropriate dose of anakinra Source: http://www.doksinet 3. Side effects a. Recognize common side effects of anakinra therapy b. Understand the studies of the safety of anakinra in childhood rheumatic diseases c. Understand the hematologic side effects of anakinra d. Understand the idiopathic adverse response to anakinra e. Understand the risk of infection with anakinra therapy f. Plan laboratory monitoring of side effects of anakinra g. Plan management of side effects of anakinra 4. Drug interactions a. Understand the significant interactions between anakinra and other medications b. Understand the usefulness of combination therapy with anakinra and other long acting immunomodulatory or immunosuppressive therapies c. Understand the potential

adverse drug interactions with combination therapy with anakinra and other long-acting immunomodulatory or immunosuppressive therapies 5. Pregnancy and lactation a. Understand the effects of anakinra therapy on pregnant women, the fetus, and in breast-feeding neonates O. Abatacept 1. Indications and contraindications a. Understand the indications for the use of abatacept in the treatment of childhood rheumatic diseases b. Understand the contraindications for the use of abatacept in the treatment of childhood rheumatic diseases 2. Pharmacology a. Understand the pharmacology of abatacept b. Understand the biologic activity of abatacept c. Prescribe the appropriate dose of abatacept 3. Side effects a. Recognize common side effects of abatacept therapy b. Understand the studies of the safety of abatacept in childhood rheumatic diseases c. Understand the idiopathic adverse response to abatacept d. Understand the risk of infection with abatacept therapy e. Plan laboratory monitoring of side

effects of abatacept f. Plan management of side effects of abatacept g. Understand studies of oncogenicity in patients with rheumatic diseases treated with abatacept 4. Drug interactions a. Understand the significant interactions between abatacept and other medications b. Understand the usefulness of combination therapy with abatacept and other long-acting immunomodulatory or immunosuppressive therapies c. Understand the potential adverse drug interactions with combination therapy with abatacept and other long-acting immunomodulatory or immunosuppressive therapies 5. Pregnancy and lactation Source: http://www.doksinet a. Understand the effects of abatacept therapy on pregnant women, the fetus, and in breast-feeding neonates P. Rituximab 1. Indications and contraindications a. Understand the indications for the use of rituximab in the treatment of childhood rheumatic diseases b. Understand the contraindications for the use of rituximab in the treatment of childhood rheumatic

diseases 2. Pharmacology a. Understand the pharmacology of rituximab b. Understand the biologic activity of rituximab c. Prescribe the appropriate dose of rituximab 3. Side effects a. Recognize common side effects of rituximab therapy b. Understand the studies of the safety of rituximab in childhood rheumatic diseases c. Understand the hematologic side effects of rituximab d. Understand the idiopathic adverse response to rituximab e. Understand the risk of infection with rituximab therapy f. Plan laboratory monitoring of side effects of rituximab g. Plan management of side effects of rituximab h. Understand studies of oncogenicity in patients with rheumatic diseases treated with rituximab 4. Drug interactions a. Understand the significant interactions between rituximab and other medications b. Understand the usefulness of combination therapy with rituximab and other long-acting immunomodulatory or immunosuppressive therapies c. Understand the potential adverse drug interactions with

combination therapy with rituximab and other long-acting immunomodulatory or immunosuppressive therapies 5. Pregnancy and lactation a. Understand the effects of rituximab therapy on pregnant women, the fetus, and in the breast-feeding neonate Q. Immunoglobulin Therapy 1. Indications and contraindication a. Know the indications for the use of intravenous immune globulin in childhood rheumatic diseases, distinguishing between proven, possible, and unproven indications b. Understand the contraindications for the use of intravenous immune globulin in childhood rheumatic diseases 2. Effectiveness in rheumatic disease a. Understand the efficacy studies of intravenous immune globulin in pediatric rheumatic diseases 3. Pharmacology a. Understand the pharmacology of intravenous immune globulin b. Understand the biologic activity of intravenous immune globulin c. Understand the method of preparation of intravenous immune globulin Source: http://www.doksinet 4. Side effects a. Recognize the

anaphylactoid-like adverse effects associated with intravenous immune globulin b. Plan management of adverse side effects of intravenous immune globulin c. Plan management to prevent the adverse effects of intravenous immune globulin d. Understand the risks of acquiring infections from intravenous immune globulin therapy 5. Drug interactions a. Recognize significant interactions between intravenous immune globulin and other drugs 6. Pregnancy and lactation a. Understand the effect of intravenous immune globulin on pregnant women and the fetus R. Mycophenolate 1. Indications and contraindications a. Understand the indications for the use of mycophenolate in the treatment of childhood rheumatic diseases b. Understand the contraindications for the use of mycophenolate in the treatment of childhood rheumatic diseases 2. Pharmacology a. Understand the pharmacology of mycophenolate b. Understand the biologic activity of mycophenolate c. Prescribe the appropriate dose of mycophenolate 3. Side

effects a. Recognize common side effects of mycophenolate therapy b. Understand the studies of the safety of mycophenolate in childhood rheumatic diseases c. Understand the hematologic side effects of mycophenolate d. Understand the idiopathic adverse response to mycophenolate e. Plan laboratory monitoring of side effects of mycophenolate f. Plan management of side effects of mycophenolate g. Understand studies of oncogenicity in patients with rheumatic diseases treated with mycophenolate 4. Drug interactions a. Understand the significant interactions between mycophenolate and other medications b. Understand the usefulness of combination therapy with mycophenolate and other long-acting immunomodulatory or immunosuppressive therapies c. Understand the potential adverse drug interactions with combination therapy with mycophenolate and other long-acting immunomodulatory or immunosuppressive therapies 5. Pregnancy and lactation a. Understand the effects of mycophenolate therapy on pregnant

women, the fetus, and in the breast-feeding neonate S. Miscellaneous Therapies 1. Understand the role of FK506 in rheumatic diseases Source: http://www.doksinet 2. 3. Understand the role of mycophenolate in rheumatic diseases Understand the role of biologic matrix components (eg, hyaluronic acid replacement) in the treatment of rheumatic diseases 4. Understand the various pharmacologic strategies in pain management for children with rheumatic diseases 5. Understand the role of dapsone in rheumatic disease 6. Recognize the side effects of dapsone 7. Recognize the need to check G6PD status before prescribing dapsone 8. Understand the role of opioids in the management of pain 9. Recognize the side effects of opioids in the management of pain 10. Understand the role of non-opioid analgesic drugs in the management of pain 11. Recognize the side effects of non-opioid analgesic drugs 12. Understand the WHO pathway for the treatment of pain 13. Understand the role of conscious sedation in

procedural pain 14. Understand the AAP guidelines for monitoring of conscious sedation 15. Understand the role of thalidomide in rheumatic diseases 16. Understand the role of pentoxifylline in rheumatic diseases T. Antihypertensive Drugs 1. Understand the various classes of antihypertensive drugs and their mechanisms of action 2. Understand the specific indications for the use of each class of antihypertensive drug 3. Understand the adverse effect commonly associated with various classes of antihypertensive drug 4. Understand drug interactions between antihypertensive drugs and drugs commonly used to treat rheumatic diseases 5. Understand the efficacy studies of angiotensin-converting enzyme inhibitors and angiotensin II receptor blocking drugs in hypertensive crisis 6. Understand the efficacy studies of angiotensin-converting enzyme inhibitors and angiotensin II receptor blocking drugs in nephritic syndrome 7. Recognize the side effects of angiotensin-converting enzyme inhibitors and

angiotensin II receptor blocking drugs 8. Understand the mechanisms of action of the angiotensin-converting enzyme inhibitors and angiotensin II receptor blocking drugs U. Antibiotic Therapy 1. Understand the indications for the use of antibiotics in immune- compromised hosts 2. Understand the contraindications for the use of antibiotics in immune- compromised hosts 3. Plan management of febrile patients with rheumatic disease receiving immunosuppressive therapy 4. Understand drug interactions involving various classes of antibiotics and antirheumatic drugs V. Vitamin and Mineral Metabolism 1. Understand current recommendations for vitamin and mineral supplementation in childhood rheumatic diseases 2. Understand the various types of vitamin D (eg, dihydroxycholecalciferol) preparations available Source: http://www.doksinet 3. 4. 5. 6. Plan monitoring and management for toxicity of vitamin D preparations Understand the efficacy studies of bisphosphonate in rheumatic diseases Know

the indications for the use of calcitonin Recognize the side effects of bisphosphonate W. Anticoagulants 1. Know the agents used and the indications for anticoagulant therapy in pediatric rheumatic diseases 2. Understand the role of warfarin in anticoagulation therapy 3. Understand the role of heparin in anticoagulation therapy 4. Understand the role of low-molecular-weight heparin in anticoagulation therapy 5. Understand the role of aspirin in anticoagulation therapy X. Gastrointestinal Agents 1. Know the agents used and the indications for the use of prokinetic agents 2. Know the agents used and the indications for the treatment of gastrointestinal irritation, inflammation, and/or ulceration Y. Emerging Therapies 1. Understand approaches to critical evaluation of experimental or unproven methods in the management of JRA 2. Recognize new strategies for the treatment of rheumatic disease currently under development and study 3. Be aware of the use of both autologous and allogeneic stem

cell therapy in autoimmune diseases Z. Alternative/Complementary Therapies 1. Be aware of the various agents recommended by proponents of alternative therapies for arthritis 2. Be aware of the side effects of alternative therapies for arthritis 3. Understand efficacy studies of fish oil in rheumatic disease IV. Musculoskeletal Pain Syndromes of Nonrheumatic Origin A. Idiopathic Pain Syndromes 1. Pain Amplification Syndromes a. Know the characteristic pain pattern in a patient with pain amplification syndrome b. Know the symptoms of pain associated with pain amplification syndrome c. Know the importance of history in pain amplification syndrome d. Know the expected physical findings in a patient with pain amplification syndrome e. Know the appropriate diagnostic evaluation in a patient with pain amplification syndrome f. Plan appropriate treatment for a patient with pain amplification syndrome g. Be aware of the role of emotional, behavioral, and psychosocial factors in pain

amplification syndromes h. Understand the importance of family history in the approach to patients with idiopathic pain syndrome 2. Growing pains/benign joint pains a. Understand the natural history of growing pains b. Know the characteristics of benign arthralgias, including hypervigilance c. Plan appropriate management of a patient with growing pains d. Recognize the signs and symptoms of growing pains Source: http://www.doksinet e. Differentiate between growing pains and other similar diseases 3. Primary fibromyalgia syndrome a. Know the criteria for diagnosing fibromyalgia b. Plan appropriate treatment for a patient with fibromyalgia c. Recognize the clinical characteristics of primary fibromyalgia d. Differentiate between fibromyalgia and other similar diseases e. Understand the prognosis for a patient with fibromyalgia f. Know the clinical criteria of chronic fatigue syndrome 4. Reflex sympathetic dystrophy (complex regional pain syndrome) a. Recognize the clinical

characteristics of reflex sympathetic dystrophy b. Recognize the characteristic changes on imaging studies of patients with reflex sympathetic dystrophy c. Know the different treatment modalities used in reflex sympathetic dystrophy d. Identify the most likely complications of reflex sympathetic dystrophy e. Understand the prognosis for a patient with reflex sympathetic dystrophy B. Pain Associated with Hypermobility 1. Fibromyalgia a. Recognize the association of hypermobility syndromes and fibromyalgia 2. Pes planus a. Recognize the signs and symptoms of pes planus b. Understand the various etiologies and differential diagnosis of pes planus c. Plan appropriate management of a patient with pes planus d. Identify the most likely complications of pes planus 3. Genu recurvatum a. Recognize the clinical characteristics of genu recurvatum 4. Recurrent Patellar Dislocation a. Recognize the clinical characteristics of recurrent patellar dislocation C. Pain Syndromes Related to Overuse 1.

Overuse syndromes a. Know the clinical characteristics associated with overuse syndromes b. Know the appropriate diagnostic evaluation of an overuse syndrome c. Know the appropriate management of an overuse syndrome 2. Patellofemoral Pain Syndrome a. Recognize the clinical characteristics of patellofemoral pain syndrome b. Know the appropriate management of patellofemoral pain syndrome 3. Plica Syndromes a. Know the clinical characteristics of plica syndromes b. Know the appropriate diagnostic evaluation of a patient with a plica syndrome c. Know the appropriate management of plica syndromes 4. Stress fractures and Ligamentous Injury a. Know the classification of ligamentous injury b. Know the appropriate management of ligamentous injury c. Understand that injury to a ligament can result in avulsion of bone d. Recognize the clinical characteristics of traumatic (stress) fracture e. Know the appropriate diagnostic evaluation of a patient with traumatic (stress) fracture Source:

http://www.doksinet f. g. Know the appropriate management of traumatic (stress) fracture Know the clinical characteristics of ligamentous injury 5. Slipped Capital Femoral Epiphysis a. Know the clinical presentation of slipped capital femoral epiphysis b. Know the appropriate diagnostic evaluation of a patient with slipped capital femoral epiphysis c. Know the appropriate management of slipped capital femoral epiphysis 6. Shin splints a. Know the clinical characteristics of shin splints b. Know the appropriate diagnostic evaluation of a patient with shin splints c. Know the appropriate management of shin splints 7. Overuse injuries affecting the upper extremity a. Know the clinical characteristics of overuse syndromes affecting the upper extremity, including those from sports participation (eg, tennis elbow, "little league" elbow, "pitchers shoulder") b. Know the appropriate diagnostic evaluation of a patient with overuse syndromes affecting the upper extremity,

including those from sports participation (eg, tennis elbow, "little league" elbow, "pitchers shoulder") c. Know the appropriate management of overuse syndromes affecting the upper extremity, including those from sports participation (eg, tennis elbow, "little league" elbow, "pitchers shoulder") 8. Tenosynovitis a. Know the clinical characteristics of tenosynovitis b. Know the appropriate diagnostic evaluation of a patient with tenosynovitis c. Know the appropriate management of tenosynovitis D. Osteochondroses 1. General a. Know the clinical characteristics of the osteochondroses, such as Koehler disease, Freiberg disease, and Thiemann disease 2. Legg-Calve-Perthes Disease a. Know the clinical characteristics of Legg-Calve-Perthes disease b. Know the long-term course of Legg-Calve-Perthes disease 3. Osgood-Schlatter disease a. Know the clinical characteristics of Osgood-Schlatter disease 4. Sinding-Larsen-Johannson syndrome a. Know the clinical

characteristics of Sinding-Larsen-Johannson syndrome 5. Scheuermann disease a. Know the clinical characteristics of Scheuermann disease E. Trauma 1. Osteochondritis dissecans a. Know the clinical characteristics associated with osteochondritis dissecans b. Know the appropriate diagnostic evaluation of osteochondritis dissecans c. Know the appropriate management of osteochondritis dissecans 2. Traumatic arthritis a. Understand the relationship of trauma to arthritis in children 3. Nonaccidental trauma Source: http://www.doksinet a. b. Know that child abuse may produce signs and symptoms of rheumatic disorders Recognize the clinical characteristics of nonaccidental trauma (child abuse) 4. Congenital indifference to pain a. Be aware of the syndromes of congenital indifference to pain in children 5. Frostbite arthropathy a. Recognize the clinical characteristics of frostbite arthropathy F. Regional Pain Syndromes Affecting the Back, Chest, or Neck 1. Differential diagnosis a. Formulate

the differential diagnosis of back pain on an anatomic basis 2. Spondylolysis and spondylolisthesis a. Know the clinical characteristics associated with spondylolysis and spondylolisthesis b. Know the appropriate management of spondylolysis and spondylolisthesis 3. Intervertebral disk herniation a. Know the clinical characteristics associated with intervertebral disk herniation b. Know the appropriate management and outcome of intervertebral disk herniation 4. Slipping rib syndrome a. Know the clinical characteristics of slipping rib syndrome 5. Costochondritis a. Know the clinical characteristics of costochondritis and Tietze syndrome 6. Torticollis a. Know the clinical characteristics of torticollis 7. Limb length discrepancy a. Identify the most likely complications of limb length discrepancy b. Know the appropriate diagnostic evaluation of limb length discrepancy c. Know the appropriate management of limb length discrepancy 8. Aneuralgic amyotrophy a. Know the clinical

characteristics of aneuralgic amyotrophy G. Erythromelalgia 1. Know the clinical characteristics associated with erythromelalgia 2. Know the appropriate diagnostic evaluation of erythromelalgia 3. Know the appropriate management of erythromelalgia 4. Differentiate between erythromelalgia and other similar diseases V. JRA and Juvenile Idiopathic Arthritis (JIA) A. Definition and Classification 1. Understand that JRA and JIA are diagnoses of exclusion 2. Recognize the criteria proposed by the American College of Rheumatology, EULAR, and ILAR for the classification of chronic arthritis in children B. Epidemiology 1. Incidence and Prevalence a. Know the estimated incidence of JRA and JIA b. Know the prevalence of JRA and JIA 2. Age of Onset a. Appreciate the differences in age of onset among the subtypes of JRA and JIA 3. Sex Ratio a. Know the sex ratios of different JIA subtypes Source: http://www.doksinet 4. Geographic and Racial Distribution a. Know the differences in incidence and

prevalence of JRA subtypes among different ethnic groups C. Etiology and Pathogenesis 1. Understand the possible contribution of infectious agents to the pathogenesis of JRA and JIA 2. Know that familial aggregation and twin concordance establish a genetic contribution to the etiology of JRA and JIA 3. Understand the current data on HLA associations with JRA and JIA subtypes 4. Understand the normal structure of the synovial tissues and the changes that occur with JIA 5. Recognize the histologic pattern of the rash of systemic JIA 6. Recognize the histologic pattern of a rheumatoid nodule 7. Recognize the range of abnormalities of synovial fluid in JIA 8. Understand the current data on the risk of developing JRA or JIA for siblings of a JRA or JIA patient 9. Understand the current data on the contribution of genes other than HLA to the pathogenesis of JRA and JIA 10. Understand the contribution of T cells to the inflammatory process of JRA and JIA 11. Understand the contribution of B

cells to the inflammatory process of JRA and JIA 12. Understand the contribution of macrophages to the inflammatory process of JRA and JIA 13. Understand the contribution of synovial cells to the inflammatory process of JRA and JIA 14. Understand the significant mediators of joint inflammation and damage in JRA and JIA 15. Understand the cytokine serum, synovial fluid, and synovial tissue profiles in various subtypes of JRA and JIA D. General Clinical Features 1. Distinguish between various causes of joint enlargement (ie, synovitis, bony enlargement, peri-articular swelling, edema) 2. Understand the mechanisms involved in chronic restriction of joint mobility 3. Understand the mechanisms involved in the acute restriction of joint mobility 4. Recognize the normal range of motion for each joint 5. Understand the current theories regarding the pathogenesis of hematuria in JRA and JIA 6. Know that patients with JIA may develop vasculitis 7. Understand the current theories of pathogenesis

of anemia in polyarticular JIA 8. Understand the usefulness of the measurement of acute phase reactants in the diagnosis and management of JIA 9. Recognize early changes apparent on x-ray studies in JRA and JIA 10. Recognize late changes apparent on x-ray studies in JRA and JIA 11. Understand the usefulness of other imaging techniques in the diagnosis and management of JRA and JIA 12. Recognize that lymphedema is a complication of JRA and JIA Source: http://www.doksinet 13. Understand current theories of the mechanism of generalized growth delay due to JRA and JIA 14. Recognize serum vitamin and protein abnormalities in a patient with JRA and JIA 15. Recognize the clinical parameters of objective arthritis 16. Understand the mechanism and time course of bony enlargement 17. Understand the mechanisms by which joint subluxation occurs in JIA 18. Understand developmental stages in childhood and their relationship to expression of pain 19. Understand the mechanisms involved in the pain

associated with synovitis 20. Recognize that the degree of expressed or perceived pain does not reflect the severity of the arthritis 21. Understand the stages of joint destruction 22. Understand the extra-articular changes secondary to persistent joint inflammation (ie, muscle atrophy, joint contracture) 23. Understand the usefulness of ultrasonography in the diagnosis and management of JRA and JIA 24. Understand the information supplied by bone scan in the diagnosis and management of JRA and JIA 25. Understand the information supplied by computed tomography in the diagnosis and management of JRA or JIA 26. Understand the information supplied by magnetic resonance imaging in the diagnosis and management of JRA and JIA 27. Recognize the clinical symptoms, radiologic findings, and treatment of Baker cyst E. General Management Principles 1. Understand the indications for and limitations of orthopedic procedures in the management of JRA or JIA: synovectomy, soft tissue surgery, and joint

replacement 2. Understand the importance of maintenance of as normal as possible a social and vocational environment in a family in whom a child has JIA 3. Understand the importance of educating teachers in the management of the child with JIA 4. Understand the psychosocial impact of JIA and its management 5. Understand treatment objectives of JIA: control of clinical manifestations, preservation of function, prevention of deformity, limitation of drug toxicity 6. Understand the value of education about arthritis for the patient with JIA and their family 7. Understand the value of a multidisciplinary approach in the treatment of JIA 8. Formulate an appropriate postoperative management plan for a patient with JIA 9. Identify and manage problems associated with school activities in a patient with JIA 10. Identify and manage problems associated with sports activities in a patient with JIA 11. Be aware of the outcome and functional status measures that are available for use in patients

with JIA 12. Be aware of the complications of JIA F. Oligoarticular JIA 1. Clinical characteristics a. Understand the prognosis of oligoarticular JIA b. Understand that oligoarticular JIA can evolve into extended oligoarticular JIA Source: http://www.doksinet c. d. e. f. g. h. i. j. Understand the diagnostic criteria for oligoarticular JIA Understand the differential diagnosis of oligoarticular JIA Understand the association of eye disease with oligoarticular JIA Know the autoantibodies found in oligoarticular JIA Understand the sex and age associations for oligoarticular JIA Understand the typical laboratory findings associated with oligoarticular JIA Understand the lack of constitutional symptoms in oligoarticular JIA Understand the pattern of joint involvement in oligoarticular JIA 2. Therapy a. Plan the use of intra-articular corticosteroids in the management of oligoarticular JIA b. Plan the pharmacotherapy of new-onset oligoarticular JIA c. Plan the pharmacotherapy of

oligoarticular JIA that has not responded to NSAIDs d. Understand the role of synovectomy in the management of oligoarticular JIA G. Polyarticular JIA 1. Clinical characteristics a. Recognize the presence and significance of subcutaneous nodules in JIA b. Understand the significance of rheumatoid factor positivity in children c. Understand the diagnostic criteria for polyarticular JIA d. Understand the differential diagnosis of polyarticular JIA e. Understand the peak age of onset for polyarticular JIA, rheumatoid factor-negative f. Understand the association of eye disease with polyarticular JIA g. Understand the sex associations for polyarticular JIA h. Know the autoantibodies found in polyarticular JIA, specifically anti- CCP and ANA i. Understand the characteristic features of rheumatoid factor-positive JIA j. Recognize the constitutional symptoms associated with polyarticular JIA k. Understand the patterns of joint involvement in polyarticular JIA l. Understand the disease course

of polyarticular JIA m. Understand the typical laboratory findings associated with polyarticular JIA n. Understand the prognosis of polyarticular JIA, rheumatoid factor-negative o. Understand the peak age of onset of polyarticular JIA, rheumatoid factor-positive p. Understand the prognosis of polyarticular JIA, rheumatoid factor-positive 2. Therapy a. Understand the efficacy studies on methotrexate in JIA b. Understand the efficacy studies of azathioprine in JIA c. Understand the efficacy studies of cyclophosphamide in JIA d. Plan the medication management of new onset polyarticular JIA e. Understand the role of low-dose corticosteroids in polyarticular JIA f. Understand the role of moderate to high-dose corticosteroids in polyarticular JIA g. Understand the indications for intra-articular corticosteroids in the management of polyarticular JIA h. Plan the medication management of polyarticular JIA that has not responded to first-line DMARDs i. Know the role of biologic agents in the

treatment of polyarticular JIA H. Systemic JIA Source: http://www.doksinet 1. Clinical characteristics a. Recognize the characteristic fever pattern of systemic JIA b. Recognize the characteristic rash of systemic JIA c. Recognize the signs and symptoms of myocarditis in systemic JIA d. Recognize the signs and symptoms of liver involvement in systemic JIA e. Understand the differential diagnosis of systemic JIA f. Understand the risk and causes of mortality in systemic JIA g. Recognize the importance of excluding malignancy in the differential diagnosis of systemic JIA h. Recognize and manage amyloidosis in a patient with JIA i. Understand the diagnostic criteria for systemic JIA j. Understand the peak age of onset for systemic JIA k. Understand the sex associations for systemic JIA l. Recognize the constitutional symptoms associated with systemic JIA m. Understand the patterns of joint involvement in systemic JIA n. Understand the different disease courses of systemic JIA o.

Recognize the signs and symptoms of pleuritis in systemic JIA p. Recognize that generalized lymphadenopathy may accompany systemic JIA q. Understand the development of macrophage activation syndrome in systemic JIA r. Recognize hematologic abnormalities associated with systemic JIA s. Understand the typical laboratory findings associated with systemic JIA t. Recognize that proinflammatory cytokine serum concentration can be increased in patients with systemic JIA u. Understand the prognosis of systemic JIA v. Know the significance of serum ferritin concentration in the management of systemic JIA 2. Therapy a. Understand the role of corticosteroids in systemic JIA b. Plan the medication management of new onset systemic JIA c. Plan the medication management of systemic JIA that has not responded to NSAIDs d. Plan management of pericarditis in systemic JIA e. Plan management of myocarditis in systemic JIA f. Plan management of pneumonitis in systemic JIA g. Plan management of severe

anemia in systemic JIA h. Plan management of macrophage activation syndrome in systemic JIA i. Plan management of failure-to-thrive in systemic JIA j. Plan management of growth delay in systemic JIA k. Understand the indications for intra-articular corticosteroids in the management of systemic JIA l. Plan the medication management of systemic JIA with arthritis that has not responded to first-line DMARDs m. Know the role of biologic agents in the treatment of systemic JIA I. Eye Disease Associated with JIA 1. Recognize the risk factors for uveitis in JIA 2. Understand inflammation of the uveal tract occurring in rheumatic diseases of children Source: http://www.doksinet 3. Understand the significance of asymptomatic disease in chronic uveitis associated with JIA 4. Recognize the signs and symptoms of chronic uveitis in the symptomatic patient with JIA 5. Understand the complications of chronic uveitis associated with JIA 6. Understand the role and limitations of topical and local

therapy in the management of chronic uveitis associated with JIA 7. Understand the current recommendations for monitoring for asymptomatic uveitis in JIA 8. Recognize the effectiveness studies of methotrexate in the treatment of iridocyclitis 9. Understand the role and limitations of systemic therapy in the management of chronic uveitis associated with JIA 10. Recognize the signs and symptoms of conjunctivitis, episcleritis, and scleritis 11. Understand the clinical differences between conjunctivitis, uveitis, episcleritis, and scleritis 12. Understand the efficacy studies of glucocorticoids in iritis 13. Understand the clinical manifestations and pathophysiology of Brown syndrome (superior oblique tenosynovitis) 14. Know the role of biologic agents in the treatment of iridocyclitis J. Complications of JIA 1. Plan the approaches to the treatment of synovial cysts 2. Plan the approaches to the treatment of tenosynovitis VI. Spondyloarthropathies A. Definition and Classification 1. Know

the classifications of the various spondyloarthropathies 2. Know the similarities of and differences among the spondyloarthropathies B. Juvenile Ankylosing Spondylitis 1. Definition and classification a. Know the diagnostic criteria for juvenile ankylosing spondylitis (eg, New York criteria, Amor Criteria, classification criteria of the European Spondyloarthropathy Study Group (ESSG), and the enthesitis- related arthritis definition of the ILAR classifica 2. Epidemiology a. Incidence and prevalence 1. Be aware of the effect of racial factors on the prevalence and genetic predisposition of juvenile ankylosing spondylitis 2. Be aware of the familial occurrence of juvenile ankylosing spondylitis 3. Know the incidence and prevalence of juvenile ankylosing spondylitis b. Age at onset 1. Know that the onset of juvenile ankylosing spondylitis is unusual in the first decade c. Sex ratio 1. Know that juvenile ankylosing spondylitis occurs predominantly in boys 3. Etiology and pathogenesis

Source: http://www.doksinet a. Know that symptoms of spondyloarthropathy can be precipitated by exposure to specific enteric pathogens (eg, Salmonella, Shigella, Yersinia, Campylobacter jejuni) b. Know the possible explanations for the linkage of HLA-B27 to expression of juvenile ankylosing spondylitis c. Understand the arthritogenic peptide hypothesis related to HLA-B27 d. Understand the role of TNF-alpha in the pathogenesis of ankylosing spondylitis 4. Genetic background a. Know that patients with spondyloarthropathy have an increased incidence of a positive HLA-B27 test b. Understand the genotype associated with juvenile ankylosing spondylitis (ie, specify which genes are associated with JAS) c. Know the chromosomal location of the gene coding for HLA-B27 antigen in juvenile ankylosing spondylitis d. Know the inheritance pattern of HLA-B27 antigen in juvenile ankylosing spondylitis e. Plan a response to requests for genetic typing of siblings, offspring, etc., in a patient with

juvenile ankylosing spondylitis f. Know that familial aggregation and twin concordance provide a genetic basis for juvenile ankylosing spondylitis g. Be aware of ethnic variation in HLA-B27 prevalence 5. Clinical manifestations a. Juvenile ankylosing spondylitis 1. Know the constitutional signs and symptoms of juvenile ankylosing spondylitis b. Arthritis 1. Differentiate the subclasses of JIA from juvenile ankylosing spondylitis based on clinical presentation 2. Know the techniques or special tests in the physical examination that indicate spine and sacroiliac involvement in patients with juvenile ankylosing spondylitis 3. Know the characteristics of the peripheral joint disease in patients with suspected juvenile ankylosing spondylitis c. Enthesitis 1. Know the anatomic definition of enthesopathy 2. Know the physical features of enthesopathy d. Extra-articular manifestations of disease 1. Juvenile ankylosing spondylitis a. Know the frequency and nature of extra-articular

manifestations in patients with juvenile ankylosing spondylitis 2. Acute iritis a. Identify the signs and symptoms characteristic of the ocular manifestations of juvenile ankylosing spondylitis 3. Cardiopulmonary disease a. Know the unique characteristics of cardiopulmonary involvement in long-standing juvenile ankylosing spondylitis 6. Diagnosis Source: http://www.doksinet a. 7. 8. 9. 10. 11. Be able to formulate a differential diagnosis in a patient with symptoms suggestive but not diagnostic of juvenile ankylosing spondylitis b. Recognize the characteristics of back pain as seen in juvenile ankylosing spondylitis c. Recognize the utility of the individual imaging modalities in juvenile ankylosing spondylitis Pathology a. Know the differences in pathology of JIA and juvenile ankylosing spondylitis Laboratory examinations a. Know which laboratory tests are associated with the diagnosis of ankylosing spondylitis (eg, blood indices, C-reactive protein, rheumatoid factor, CCP,

ANA, complement, synovial fluid analysis) Radiologic changes a. Interpret the characteristics on findings on imaging studies in enthesopathy b. Know the early and late radiologic features of juvenile ankylosing spondylitis and the time frame involved c. Know the role of magnetic resonance imaging in the diagnosis of juvenile ankylosing spondylitis Treatment a. Anti-inflammatory, anti-microbial, and second-line 1. Know the different categories of drugs most commonly used in the treatment of juvenile ankylosing spondylitis 2. Know the treatment approach to the eye disease of juvenile ankylosing spondylitis 3. Know the biologic agents used in the treatment of juvenile ankylosing spondylitis 4. Know the role of thalidomide in the treatment of juvenile ankylosing spondylitis 5. Know the role of bisphosphonates in the treatment of juvenile ankylosing spondylitis b. Physical and occupational therapy 1. Know the unique aspects of physical therapy applied to juvenile ankylosing spondylitis c.

Surgery 1. Be aware of the indications for joint replacement in a patient with juvenile ankylosing spondylitis 2. Know the postoperative problems in patients with juvenile ankylosing spondylitis Disease course and prognosis a. Know the prognosis for the development of peripheral joint disease in patients with juvenile ankylosing spondylitis b. Be aware of the clinical course and prognosis in a patient with juvenile ankylosing spondylitis c. Recognize the complications of juvenile ankylosing spondylitis d. Know that peripheral joint disease can precede axial disease in juvenile ankylosing spondylitis Source: http://www.doksinet C. Enthesitis-Related JIA and Seronegative Enthesitis and Arthritis 1. Definition and classification 2. Epidemiology a. Know the epidemiologic features of enthesitis-related JIA 3. Genetic background a. Know the familial background and HLA association in some patients with enthesitis-related JIA 4. Clinical manifestations a. Recognize the clinical

manifestations of enthesitis-related JIA 5. Diagnosis a. Distinguish enthesitis-related JIA from juvenile ankylosing spondylitis 6. Laboratory examination a. Know the laboratory findings associated with enthesitis-related JIA 7. Radiologic changes a. Recognize the radiologic features of enthesitis-related JIA 8. Treatment a. Develop an appropriate management plan for a patient with enthesitis-related JIA 9. Disease course and prognosis a. Know the course and prognosis of enthesitis-related JIA b. Know the percentage of patients with enthesitis-related JIA that develop other spondyloarthropathies, including ankylosing spondylitis D. Psoriatic JIA 1. Definition and classification a. Know the criteria for the diagnosis of juvenile psoriatic arthritis proposed by the ILAR criteria (Edmonton revision) 2. Epidemiology a. Incidence and prevalence 1. Know the incidence and prevalence of psoriatic JIA b. Age at onset and sex ratio 1. Know the age at onset and sex ratio of psoriatic JIA 3.

Etiology and pathogenesis a. Be aware of current theories about the etiology and pathogenesis of psoriatic JIA 4. Genetic background a. Know the genetic linkage between HLA antigens and certain features of psoriatic JIA 5. Clinical manifestations a. Arthritis and enthesitis 1. Know the pattern of joint involvement in a patient with psoriatic JIA b. Extra-articular manifestations of disease 1. Skin a. Recognize the dermatologic features associated with psoriatic JIA 2. Nails a. Recognize the earliest changes in the nails seen in children with psoriatic JIA 3. Uveitis a. Know the presentations and course of uveitis in psoriatic JIA Source: http://www.doksinet 6. Diagnosis a. General physical examination b. Musculoskeletal examination 1. Formulate a differential diagnosis in a patient with symptoms suggestive but not diagnostic of psoriatic JIA 7. Pathology a. Recognize the histopathologic features of psoriatic JIA 8. Laboratory examinations a. Know the laboratory findings in

psoriatic JIA 9. Radiologic changes a. Know the unique characteristics of the radiologic changes in psoriatic JIA 10. Treatment a. Know the advanced drug therapy for recalcitrant psoriatic JIA b. Understand the role of biologics in the treatment of psoriatic JIA 11. Disease course and prognosis a. Know the courses of the different clinical forms of psoriatic JIA E. Inflammatory Bowel Disease 1. Definition and classification a. Understand the nature of the seronegative arthropathies that accompany the inflammatory bowel diseases 2. Epidemiology a. Incidence and prevalence 1. Know the frequency of arthritis in patients with inflammatory bowel disease b. Age at onset and sex ratio 1. Know the age of onset and sex ratio in patients with inflammatory bowel disease 3. Etiology and pathogenesis a. Know the theories for extra-intestinal manifestations of arthritis associated with inflammatory bowel disease 4. Genetic background a. Know the relationship between HLA-B27 and certain aspects of

arthritis associated with inflammatory bowel disease 5. Clinical manifestations a. Arthritis and enthesitis 1. Recognize the pattern of joint pain and joint involvement in a patient with arthritis associated with inflammatory bowel disease b. Extra-articular manifestations of disease 1. General a. Recognize the signs and symptoms of inflammatory bowel disease in addition to the arthritis-related manifestations 2. Hepatic disease a. Know the hepatic features in patients with inflammatory bowel disease 3. Gastrointestinal disease a. Know the gastrointestinal features in patients who have arthritis associated with inflammatory bowel disease Source: http://www.doksinet b. Know the relationship between bowel symptoms and joint symptoms in a patient with arthritis associated with inflammatory bowel disease 4. Cutaneous a. Identify the histopathologic features in patients with arthritis associated with inflammatory bowel disease 6. Diagnosis and laboratory examinations a. Formulate a

differential diagnosis for a patient with inflammatory bowel disease and associated joint symptoms b. Perform an appropriate laboratory evaluation of a patient with inflammatory bowel disease and associated joint symptoms 7. Pathology a. Identify histopathologic features of arthritis associated with inflammatory bowel disease 8. Radiologic changes a. Recognize the radiologic findings of inflammatory bowel disease in a patient with associated joint symptoms 9. Treatment a. Prescribe the appropriate drug therapy for a patient with arthritis associated with inflammatory bowel disease b. Know the appropriate nutritional management of a patient with arthritis associated with inflammatory bowel disease c. Be aware of current therapy for the management of inflammatory bowel disease 10. Disease course and prognosis a. Know the course and prognosis of arthritis associated with inflammatory bowel disease b. Recognize the complications of arthritis associated with inflammatory bowel disease F.

Reactive Arthritis 1. Definition and classification a. Know the differences between endemic and epidemic reactive arthritis b. Formulate a differential diagnosis in a patient with arthritis, conjunctivitis, and pyuria 2. Epidemiology a. Know the reasons for the perceived male predominance of patients with reactive arthritis 3. Etiology and pathogenesis a. Know the role of environmental factors in the expression of reactive arthritis b. Be aware of the causative agents of arthritis following infection with enteric bacteria: Shigella, Yersinia, Salmonella, Campylobacter c. Be aware of the association of arthritis with Mycoplasma infections d. Understand the role of persistent Chlamydia trachomatis infection of the synovium in reactive arthritis 4. Genetic background a. Know the relationship between HLA-B27 and reactive arthritis 5. Clinical manifestations Source: http://www.doksinet a. Recognize the clinical manifestations of reactive arthritis: ocular, mucocutaneous, genitourinary,

articular 6. Laboratory examinations a. Interpret the laboratory findings in a patient with reactive arthritis 7. Pathology a. Identify the histopathologic features of reactive arthritis 8. Radiologic changes a. Recognize the radiologic changes associated with reactive arthritis 9. Treatment a. Know the appropriate management of reactive arthritis 10. Disease course and prognosis a. Know the course and prognosis of reactive arthritis VII. Systemic Lupus Erythematosus A. Definition and Classification 1. Recognize the American College of Rheumatology criteria for the classification of SLE 2. Recognize how the prognosis of SLE has changed over the past few decades 3. Understand the historical development of tests for SLE B. Epidemiology 1. Incidence and prevalence a. Know the incidence and prevalence of SLE in children b. Know the incidence and prevalence of SLE in adults 2. Age of onset a. Know the patterns of age of onset of SLE 3. Sex ratio a. Know the ratio of males to females

affected by SLE and the factors that affect it 4. Geographic and racial distribution a. Know the racial implications for the severity and prognosis of SLE b. Know the variations in incidence and prevalence of SLE in different populations according to race and geographic region C. Etiology and Pathogenesis 1. Autoimmunity a. Understand current theories about the causes of SLE in animals and humans b. Understand the current theories regarding the role of infectious agents in SLE c. Understand the current theories of development of autoimmunity to nuclear antigens d. Understand the role of T cells in the development of autoantibodies e. Understand the role of autoantibodies to nuclear antigens in the development of the manifestations of SLE f. Recognize which autoantibodies have been demonstrated to cause tissue damage in SLE g. Understand the increased incidence of antinuclear antibodies in family members of patients with SLE h. Understand the current theories regarding the development

of antiphospholipid antibodies Source: http://www.doksinet i. Understand the autoimmune differentiation between organ-specific disease and multisystem disease, and the participation of anti-tissue antibodies in each 2. Hormonal factors a. Recognize the contribution of sex hormones to the initiation and perpetuation of SLE in animal models b. Understand the role of exogenous and endogenous sex hormones in human SLE, including the effects of oral contraceptives, pregnancy, and postpartum hormonal changes 3. Immune-complex disease a. Understand the possible triggers and mechanisms of immune complex formation b. Understand how nucleic acid-containing immune complexes trigger inflammation 4. Genetic background a. Understand the increased incidence of autoimmunity in relatives of patients with SLE b. Understand the concordance and discordance of SLE in identical twins c. Understand the HLA and nonHLA genetic factors associated with SLE d. Know that C2 deficiency is associated with SLE e.

Know that C4 deficiency syndromes are associated with SLE f. Understand the ethnic contribution to the incidence and clinical characteristics of SLE g. Be aware of the increased incidence of SLE in Klinefelter syndrome 5. Environmental factors and drug-induced SLE a. Recognize that ultraviolet light plays a role in the induction and flare of skin disease in SLE b. Recognize the effects of diet and other environmental factors in the initiation of SLE c. Understand which medications are associated with the induction of drug-induced SLE d. Know the clinical and laboratory features that differentiate drug-induced from idiopathic SLE e. Understand the proposed mechanisms of induction of drug- induced SLE f. Understand the prognosis of drug-induced SLE D. Pathology 1. Skin a. Recognize the histologic patterns of skin involved in SLE b. Understand the histology and significance of the lupus band test c. Recognize the histology of discoid lupus 2. Renal a. Understand the histologic pattern of

mesangial lupus nephritis b. Understand the immunofluorescent patterns on mesangial lupus nephritis c. Understand the histologic patterns of focal proliferative glomerulonephritis d. Understand the histologic patterns of diffuse proliferative glomerulonephritis e. Understand the immunofluorescent pattern of diffuse proliferative glomerulonephritis f. Understand the histologic patterns of membranous lupus glomerulonephritis Source: http://www.doksinet g. Understand the immunofluorescent patterns of membranous lupus glomerulonephritis h. Understand the histologic patterns of interstitial nephritis in SLE i. Understand the histologic patterns of glomerulosclerosis in SLE j. Understand the histologic patterns of necrotizing angiitis in SLE k. Understand the activity and chronicity indices for lupus nephritis l. Understand the electron microscopic patterns of the various types of lupus nephritis m. Understand the renal-specific prognosis of each of the WHO classifications of nephritis

3. Other organ systems a. Understand the histologic pattern in patients with SLE and cardiac valve involvement (Libman-Sacks endocarditis) b. Understand the histologic patterns in patients with SLE and blood vessel involvement c. Understand the immunofluorescent patterns of vasculitis in SLE d. Identify bone marrow pathology in patients with SLE e. Understand the histologic patterns of lymphoid tissue in patients with SLE E. Clinical Manifestations 1. Clinical presentation a. Recognize the variability of the patterns of clinical presentation of SLE in childhood b. Identify differences between adult and pediatric SLE 2. Mucocutaneous a. Recognize the mucocutaneous manifestations of SLE b. Recognize discoid lesions of lupus c. Recognize lesions of subacute cutaneous lupus 3. Musculoskeletal a. Recognize the characteristics of arthritis associated with SLE b. Recognize the characteristics of myositis associated with SLE c. Recognize the characteristics of avascular necrosis associated

with SLE 4. Vascular a. Recognize the characteristics of vasculitis affecting skin and internal organs in childhood SLE b. Understand hypertension in SLE c. Understand the current theories regarding the development of arteriosclerosis in SLE 5. Cardiac a. Understand the characteristics of endocarditis in SLE b. Recognize the signs and symptoms of myocarditis in SLE c. Recognize the signs and symptoms of pericarditis in SLE 6. Pulmonary a. Understand the various types of pulmonary manifestations of SLE b. Recognize the signs and symptoms of pneumonitis and pleural effusions in SLE c. Recognize the signs and symptoms of pulmonary fibrosis in SLE d. Recognize the signs and symptoms of pulmonary hemorrhage in SLE e. Recognize the signs and symptoms of pulmonary hypertension in SLE Source: http://www.doksinet f. g. Know the appropriate management of pulmonary involvement from SLE Recognize the differences in SLE pulmonary disease versus opportunistic infection 7. Gastrointestinal a.

Understand the signs and symptoms of gastrointestinal involvement in SLE b. Understand the signs and symptoms of hepatic involvement in SLE 8. Lymphoid system a. Recognize the characteristics of lymphoid system involvement in SLE 9. Endocrine and exocrine glands a. Recognize the characteristics of endocrine gland involvement in SLE b. Recognize the characteristics of thyroid involvement in SLE c. Recognize the characteristics of pancreatic involvement in SLE d. Understand the role of autoantibodies in the involvement of the thyroid in SLE 10. Nervous system a. Recognize the signs and symptoms of central nervous system involvement in SLE b. Understand the various types of nervous system involvement in SLE c. Recognize the signs and symptoms of peripheral nerve involvement in SLE d. Recognize the signs and symptoms of transverse myelitis in SLE e. Recognize the neuropsychiatric signs and symptoms of SLE f. Understand the treatment of depression in SLE 11. Renal a. Understand the causes

of renal involvement in SLE b. Recognize the significance and various types of renal involvement in SLE c. Understand the long-term studies of outcome of renal disease in patients with SLE d. Understand the predictors of progressive renal disease in patients with SLE e. Understand the correlations between clinical renal disease and renal histology in patients with SLE 12. Hematologic a. Recognize the signs and symptoms of hematologic involvement in SLE b. Understand the various types of hematologic involvement in SLE c. Understand coagulopathies associated with SLE d. Recognize the signs and symptoms of antiphospholipid antibody syndrome in SLE F. Laboratory Examinations 1. Inflammation a. Understand the significance and difference between elevated sedimentation rates and elevated C-reactive protein in patients with SLE 2. Hematologic abnormalities a. Interpret results of tests to detect hematologic abnormalities in patients with SLE b. Understand the mechanism of the hematologic

abnormalities in patients with SLE 3. Autoantibodies a. Antinuclear antibodies 1. Understand the indications for specific antinuclear antibody tests in patients with SLE (eg, double-stranded DNA, anti-Ro/La, anti-Sm, anti-histone, anti-RNP) 2. Interpret results of extractable nuclear antibody tests in patients with SLE 3. Understand and interpret the different ways to measure autoantibodies in SLE 4. Interpret the results of anti-ds DNA antibodies in patients with SLE Source: http://www.doksinet 5. Interpret the results of anti-histone antibodies in patients with SLE b. Antiphospholipid antibodies 1. Understand the indications for antiphospholipid antibody tests in patients with SLE 2. Interpret results of antiphospholipid antibody tests in patients with SLE c. Other autoantibodies (eg, thyroid, anti-neuronal, insulin) 1. Understand the indications for tests to detect other autoantibodies (eg, thyroid, anti-neuronal, insulin) in patients with SLE 2. Interpret results of tests to

detect other autoantibodies (eg, thyroid, anti-neuronal, insulin) in patients with SLE 4. Complement a. Know how to test for complement activation fragments b. Understand the indications for specific serum complement component tests in patients with SLE c. Interpret the results of specific serum complement component tests in patients with SLE d. Understand the phenotype of SLE in patients with complement deficiencies 5. Synovial fluid a. Understand the indications for synovial fluid analysis in patients with SLE 6. Urine a. Understand the indications for the various types of urine studies in patients with SLE b. Interpret the results of various urine studies in patients with SLE 7. Cerebrospinal fluid a. Understand the indications for examination of the cerebrospinal fluid in patients with SLE b. Interpret the results of examination of the cerebrospinal fluid in patients with SLE c. Understand the types of cerebrospinal fluid analyses useful in SLE 8. Pleural and pericardial fluid a.

Understand the indications for examination of the pleural/pericardial fluid in patients with SLE b. Interpret the results of examination of the pleural/pericardial fluid in patients with SLE 9. Lipoprotein analysis a. Understand the indications for serum lipoprotein analysis in patients with SLE b. Interpret the results of serum lipoprotein analysis in patients with SLE c. Understand the effects of medications on serum concentrations of lipoproteins G. Other Diagnostic Techniques 1. Understand the various diagnostic studies that are useful in identifying and managing central nervous system involvement in patients with SLE 2. Understand the various diagnostic studies that are useful in identifying and managing pulmonary involvement in patients with SLE 3. Understand the various diagnostic studies that are useful in identifying and managing cardiac involvement in patients with SLE 4. Understand the various diagnostic studies that are useful in identifying and managing renal involvement

in patients with SLE Source: http://www.doksinet 5. Interpret the results of studies that may be useful in managing central nervous system involvement in patients with SLE 6. Interpret the results of pulmonary function studies in patients with SLE 7. Interpret the results of studies that may be useful in managing cardiac involvement in patients with SLE 8. Interpret the results of renal imaging and functional studies in patients with SLE H. Treatment 1. General measures a. Sunscreens 1. Understand current modalities to protect against exposure to ultraviolet light in patients with SLE 2. Understand how exposure to artificial sources of ultraviolet light is a risk in patients with SLE b. Immunizations 1. Understand immunization recommendations for children with SLE c. Diet 1. Understand appropriate dietary management of patients with SLE 2. Non-steroidal anti-inflammatory drugs a. Understand the indications for low-dose salicylates in the management of childhood SLE b. Understand

the indications for NSAIDs in the management of childhood SLE c. Understand the complications of NSAIDs when used to treat childhood SLE 3. Antimalarial drugs a. Understand the indications for antimalarial drugs in the management of childhood SLE b. Understand the effectiveness of hydroxychloroquine therapy in childhood SLE c. Understand the complications of hydroxychloroquine therapy in childhood SLE 4. Corticosteroids a. Low-dose therapy 1. Understand the indications for low-dose corticosteroid therapy in childhood SLE 2. Understand the efficacy studies of low-dose glucocorticoids in SLE b. High-dose therapy 1. Understand the indications for high-dose corticosteroid therapy in childhood SLE 2. Understand the complications and disadvantages of high-dose corticosteroid therapy when used to treat childhood SLE c. Alternate-day corticosteroids 1. Understand the indications for alternate-day corticosteroid therapy in childhood SLE d. Pulse therapy 1. Understand the indications for pulse

corticosteroid therapy in childhood SLE 2. Know the complications and disadvantages of pulse corticosteroid therapy when used to treat childhood SLE 5. Immunosuppressive/immunomodulatory agents a. Cyclophosphamide Source: http://www.doksinet 1. 2. 3. Understand the efficacy studies of cyclophosphamide in children with SLE Understand the efficacy studies of cyclophosphamide in adults with SLE Understand the indications for the daily use of cyclophosphamide in the management of childhood SLE 4. Understand the complications and disadvantages of cyclophosphamide in the management of childhood SLE 5. Understand the indications for pulse cyclophosphamide in the management of childhood SLE 6. Understand the effectiveness of pulse cyclophosphamide in the management of childhood SLE b. Azathioprine 1. Understand the indications for the use of azathioprine in the management of childhood SLE 2. Understand the effectiveness of azathioprine in the management of childhood SLE 3. Understand the

complications and disadvantages of azathioprine in the management of childhood SLE c. Mycophenolate mofetil 1. Understand the indications for the use of mycophenolate mofetil in the management of childhood SLE 2. Understand the effectiveness of mycophenolate mofetil in the management of childhood SLE 3. Understand the complications and disadvantages of mycophenolate mofetil in the management of childhood SLE d. Methotrexate 1. Understand the indications for the use of methotrexate in the management of childhood SLE 2. Understand the effectiveness of methotrexate in the management of childhood SLE 3. Understand the complications and disadvantages of methotrexate in the management of childhood SLE e. Cyclosporine 1. Understand the indications for the use of cyclosporine in the management of childhood SLE 2. Understand the effectiveness of cyclosporine in the management of childhood SLE 3. Understand the complications and disadvantages of cyclosporine in the management of childhood SLE f.

Other 1. Understand the indications for the use of combination therapy in the management of childhood SLE 2. Understand the limitations of autologous stem cell transplantation in SLE 3. Understand common antihypertensive medication use in SLE 6. Plasmapheresis/apheresis a. Understand the effectiveness of plasmapheresis/apheresis in the management of childhood SLE Source: http://www.doksinet 7. Dialysis and renal transplantation a. Understand the indications for dialysis in the management of childhood SLE b. Understand the indications for and long-term prognosis of renal transplantation in childhood SLE c. Understand management of patients with SLE who have undergone renal transplantation d. Understand the long-term prognosis for patients with SLE on chronic dialysis e. Understand the recurrence rate of lupus nephritis in donor kidneys 8. Experimental therapies a. Understand the role of hormone-based therapies in systemic lupus erythematosus 9. Surgical therapy a. Understand the

indications for and prevention of complications from splenectomy in SLE b. Understand the role of total joint replacements in the management of avascular necrosis I. Course and Prognosis 1. Morbidity and mortality a. Understand the causes of morbidity in SLE, including iatrogenic complications b. Know the mortality rates in childhood and adult SLE c. Understand the most common causes of death in patients with SLE d. Understand the risk factors for myocardial infarction in patients with SLE e. Understand the psychosocial issues in children with SLE, including compliance issues 2. Pregnancy and postpartum complications a. Understand the risks and complications of pregnancy in patients with SLE b. Understand the management of a pregnant patient with SLE c. Recognize the risk of postpartum exacerbations of SLE d. Understand the management of postpartum exacerbations of SLE e. Understand management of a pregnant patient with SLE and antiphospholipid antibodies 3. Natural history of

untreated disease a. Understand the natural history of untreated SLE b. Understand the management of asymptomatic patients with SLE and serologic abnormalities 4. Outcome measures a. Be aware of the various instruments that can be used to measure functional status in SLE J. Neonatal Lupus Syndrome 1. General a. Understand that infants of mothers with SLE are at risk for neonatal lupus syndromes b. Understand the risk for subsequent children to have neonatal lupus syndrome 2. Clinical manifestations a. Recognize the clinical manifestations of neonatal SLE syndromes, including cardiac, hematocytopenia, hepatic involvement b. Recognize the cutaneous manifestations of neonatal SLE syndromes Source: http://www.doksinet 3. Pathogenesis a. Understand the pathogenesis of the neonatal lupus syndromes, including the role of specific autoantibodies 4. Outcomes a. Understand the natural history of neonatal SLE 5. Indications for intervention a. Understand the indications for intervention in

neonatal lupus syndromes, including conducting defects and hematocytopenias 6. Management a. Understand the management options, their risks, and advantages in neonatal lupus syndromes, including cesarean delivery, exchange transfusion, corticosteroids, external cardiac pacing b. Plan monitoring for detection of neonatal lupus syndromes during gestation c. Understand the prenatal and postnatal management of serious manifestations of neonatal lupus syndromes VIII. Juvenile Dermatomyositis A. Definition and Classification 1. Understand the criteria of Bohan and Peter for the classification of dermatomyositis B. Epidemiology 1. Incidence and prevalence a. Understand the incidence and prevalence of juvenile dermatomyositis 2. Age at onset a. Be familiar with the range of age of onset of juvenile dermatomyositis 3. Sex ratio a. Recognize that juvenile dermatomyositis occurs twice as often in girls as in boys 4. Geographic and racial distribution 5. Other a. Be familiar with the seasonal

incidence of juvenile dermatomyositis b. Be aware that there is no increased risk of malignancy in children with juvenile dermatomyositis C. Etiology and Pathogenesis 1. Pathogenesis a. Understand the current theories about the pathogenesis of juvenile dermatomyositis b. Be aware that ultraviolet light play a role in the induction and flare of skin disease in juvenile dermatomyositis 2. Familial dermatomyositis 3. Genetic relationships a. Understand the common genetic associations with juvenile dermatomyositis 4. Other a. Understand the relationship of immunodeficiency to juvenile dermatomyositis b. Understand the role of infection in the etiology of juvenile dermatomyositis D. Clinical Features 1. Constitutional signs and symptoms 2. Muscle disease a. Recognize the classic pattern of muscle weakness in juvenile dermatomyositis Source: http://www.doksinet b. Recognize the implications of involvement of the respiratory and palatopharyngeal muscles in juvenile dermatomyositis c. Be

familiar with the Medical Research Councils grading system for muscle strength 3. Other musculoskeletal abnormalities 4. Cutaneous disease a. Recognize the classic dermatologic manifestations of juvenile dermatomyositis (rash, edema, etc) b. Understand the implications of skin ulcers in juvenile dermatomyositis c. Be aware of the concept of amyopathic dermatomyositis d. Understand the relationship and implications of the presence of acanthosis nigricans to autoimmune and rheumatic diseases 5. Calcinosis a. Recognize the risk factors in development of calcinosis in juvenile dermatomyositis b. Recognize the possible complications of calcinosis 6. Oral, mucosal, and gastrointestinal disease a. Recognize the signs and symptoms of vasculitis of the gastrointestinal tract in juvenile dermatomyositis 7. Other a. Recognize the ocular manifestations of juvenile dermatomyositis b. Recognize the cardiac manifestations of juvenile dermatomyositis c. Recognize the pulmonary manifestations of

juvenile dermatomyositis E. Differential Diagnosis 1. Postinfectious myositis a. Differentiate postinfectious myositis from juvenile dermatomyositis in etiology: muscles involved, prognosis, therapy 2. Neuromuscular diseases and myopathies a. Differentiate juvenile dermatomyositis from neuromuscular diseases and myopathies 3. Myositis with other connective tissue diseases a. Differentiate juvenile dermatomyositis from myositis occurring as part of other connective tissue diseases 4. Miscellaneous disorders a. Differentiate juvenile dermatomyositis from other disorders such as rhabdomyolysis, fibrodysplasia ossificans progressiva, pyomyositis F. Pathology 1. Skeletal muscle a. Recognize histopathologic changes occurring in the skeletal muscle of patients with juvenile dermatomyositis 2. Blood vessels a. Recognize histopathologic changes occurring in the vascular components of patients with juvenile dermatomyositis 3. Connective tissues a. Recognize histopathologic changes occurring in

the connective tissues of patients with juvenile dermatomyositis Source: http://www.doksinet 4. Skin a. Recognize histopathologic changes occurring in the skin of patients with juvenile dermatomyositis 5. Gastrointestinal tract a. Recognize histopathologic changes occurring in the gastrointestinal tract of patients with juvenile dermatomyositis 6. Heart a. Recognize histopathologic changes occurring in the heart of patients with juvenile dermatomyositis 7. Kidneys a. Recognize histopathologic changes occurring in the kidneys of patients with juvenile dermatomyositis 8. Bones a. Recognize the association of osteopenia and inflammatory myopathy G. Diagnostic Examinations 1. Evaluation a. Plan the diagnostic evaluation of a child with juvenile dermatomyositis 2. Autoantibodies a. Interpret autoantibody test results in a patient with juvenile dermatomyositis b. Recognize the significance of the presence of myositis-specific antibodies in some patients with juvenile dermatomyositis (eg,

anti-synthetase, anti-Mi2, anti-signal recognition peptides, Jo-1) 3. Muscle enzymes a. Creatine kinase 1. Understand the relationship of creatine kinase to disease activity in juvenile dermatomyositis b. Transaminases 1. Interpret the significance of increased transaminase activities in juvenile dermatomyositis c. Aldolase 1. Understand the relationship of aldolase to disease activity in juvenile dermatomyositis d. Lactic dehydrogenase 1. Interpret the significance of increased lactic dehydrogenase activity in juvenile dermatomyositis and other connective tissue diseases 4. Electromyography a. Recognize patterns of electromyographic findings in juvenile dermatomyositis 5. Muscle biopsy a. Identify the characteristic histopathologic changes associated with the worst prognosis in juvenile dermatomyositis 6. Other laboratory tests a. Understand the role of von Willebrand (Factor VIII) antigen measurement in juvenile dermatomyositis b. Understand the significance of surrogate markers of

disease activity in patients with juvenile dermatomyositis (ie, neopterin, percentage of B cells, etc) H. Radiologic Changes Source: http://www.doksinet 1. Understand the role of MRI including specific techniques in evaluating muscle disease, including inflammatory myopathy 2. Recognize the radiologic changes of the extraskeletal systems seen on x-ray studies in patients with juvenile dermatomyositis 3. Understand the role of technetium bone scan in patients with juvenile dermatomyositis I. Treatment 1. General supportive care 2. Physical and occupational therapy a. Understand the role of physical and occupational therapy in patients with juvenile dermatomyositis who have active and inactive disease 3. Glucocorticoid drugs a. Understand the doses and routes of administration of glucocorticoid drug regimens commonly used in patients with juvenile dermatomyositis b. Understand the efficacy studies of glucocorticoids in juvenile dermatomyositis 4. Other therapy a. Understand the

effectiveness studies of methotrexate in children with juvenile dermatomyositis syndrome b. Understand the indications and contraindications for use of methotrexate, cyclosporin, hydroxychloroquine, and cyclophosphamide in juvenile dermatomyositis c. Recognize the efficacy studies of hydroxychloroquine in juvenile dermatomyositis 5. Intravenous immunoglobulin a. Understand the indications for using intravenous immunoglobulin in juvenile dermatomyositis, the usual dose used, and expected complications b. Understand the efficacy studies of intravenous IgG in dermatomyositis 6. Management of calcinosis a. Understand the potential complications of calcinosis and be able to state a management plan for each (infection, pain, swelling, decreased range of motion, etc) J. Disease Course and Prognosis 1. Functional outcome a. Understand the variability in the disease course in juvenile dermatomyositis, including the natural history of untreated disease b. Recognize the factors affecting outcome

in juvenile dermatomyositis: delayed treatment, characteristics of disease, amount of muscle destruction, drug toxicity 2. Death a. Understand the causes of death in juvenile dermatomyositis IX. Vasculitis A. Definition and Classification 1. Know the characteristic pathology of the various vasculitides 2. Know the variables used to classify vasculitis in children, including size of vessel, type of cellular infiltrate, organ system involvement, and syndromic presentation B. Polyarteritis 1. Polyarteritis nodosa a. Epidemiology Source: http://www.doksinet 1. Know the epidemiologic features of polyarteritis nodosa, including the role of preceding infection b. Etiology 1. Know the role of immune complexes in the etiology of necrotizing vasculitis 2. Understand the role of endothelial and inflammatory cell antigens and their ligands in the pathogenesis of necrotizing vasculitis 3. Understand the potential mechanisms by which various infections may cause necrotizing vasculitis 4. Know

the associations of drugs in the etiology of necrotizing vasculitis 5. Know the associations of recreational drugs in the etiology of necrotizing vasculitis c. Clinical manifestations 1. Recognize the common clinical manifestations of polyarteritis nodosa, including central and peripheral nervous system and renal manifestations d. Diagnosis 1. Plan an appropriate diagnostic evaluation in the assessment of a patient with polyarteritis nodosa e. Pathology 1. Recognize the characteristic histopathologic changes associated with polyarteritis nodosa f. Radiographic changes 1. Recognize the characteristic radiographic changes associated with polyarteritis nodosa g. Laboratory findings 1. Recognize the common laboratory findings in polyarteritis nodosa 2. Understand the role of nerve conduction testing in vasculitis 3. Understand the role of biopsy in establishing the diagnosis of vasculitis h. Treatment 1. Plan the medical management of a patient with polyarteritis nodosa 2. Understand the

efficacy studies of cyclophosphamide in children and adults with vasculitis 3. Understand the efficacy studies of glucocorticoids in polyarteritis i. Disease course and prognosis 1. Know the disease course of polyarteritis nodosa 2. Identify the complications of polyarteritis nodosa 2. Kawasaki syndrome a. Epidemiology 1. Incidence and prevalence a. Know the putative epidemiologic associations with certain epidemics of Kawasaki disease 2. Age of onset a. Know that the frequency of Kawasaki disease is increased in children younger than 3 years of age 3. Sex ratio a. Know that Kawasaki disease is more common in boys than in girls 4. Geographic and racial distribution Source: http://www.doksinet a. Be aware that Kawasaki syndrome is more common in children of Asian origin b. Etiology and pathogenesis 1. Understand current concepts regarding the etiology and pathogenesis of Kawasaki disease 2. Know the abnormalities of production of inflammatory mediators in Kawasaki disease 3. Know

the possible role of infection in the pathogenesis of Kawasaki disease c. Clinical features 1. Know the diagnostic criteria for Kawasaki disease 2. Know the risk factors for coronary artery involvement in Kawasaki disease 3. Recognize the characteristic features of each phase of Kawasaki disease 4. Recognize the cardiac manifestations of each phase of Kawasaki disease 5. Recognize the skin manifestations of each phase of Kawasaki disease 6. Recognize the gastrointestinal manifestations of Kawasaki disease 7. Recognize the central nervous system manifestations of Kawasaki disease 8. Know the characteristics of the fever pattern associated with Kawasaki disease 9. Recognize the ocular manifestations of each phase of Kawasaki disease, including uveitis and conjunctival injection 10. Know the diagnostic criteria for incomplete Kawasaki disease d. Diagnosis 1. Know the echocardiographic findings of Kawasaki disease 2. Know the role of arteriography in evaluating a patient with Kawasaki

disease 3. Know the role of imaging modalities in Kawasaki disease e. Pathology f. Laboratory findings 1. Identify the characteristic laboratory findings in a patient with Kawasaki disease g. Treatment 1. Plan the medical management of a patient with Kawasaki disease, including the role and timing of intravenous immune globulin, the use of aspirin, the use of glucocorticoids, and other modalities a. Understand the efficacy studies of glucocorticoids in Kawasaki disease b. Plan the medical management of a patient with Kawasaki disease in whom intravenous immune globulin and corticosteroids have failed h. Disease course and prognosis 1. Know the frequency of complications in Kawasaki disease 2. Know the mortality rate in Kawasaki disease, and factors affecting it 3. Know long-term outcome for children with Kawasaki disease 4. Know the purported relationship of Kawasaki disease to infantile polyarteritis nodosa 3. Microscopic polyangiitis a. Clinical features 1. Recognize the clinical

manifestations of microscopic polyangiitis 2. Know the similarities between microscopic polyangiitis and granulomatosis with polyangiitis (Wegener granulomatosis) Source: http://www.doksinet b. Laboratory findings 1. Identify the common laboratory findings of microscopic polyangiitis 2. Recognize the finding of hemosiderin-laden macrophages in pulmonary hemorrhage in microscopic polyangiitis and granulomatosis with polyangiitis (Wegener granulomatosis) c. Treatment 1. Plan the medical management of patients with microscopic polyangiitis d. Outcome 1. Know the disease course of patients with microscopic polyangiitis 4. Cutaneous polyarteritis nodosa a. Clinical features 1. Recognize the clinical manifestations of cutaneous polyarteritis nodosa 2. Recognize the relationship between group A streptococcal infections and cutaneous polyarteritis nodosa b. Laboratory findings 1. Identify the common laboratory findings of cutaneous polyarteritis nodosa c. Treatment 1. Plan the medical

management of patients with polyarteritis nodosa d. Outcome 1. Know the disease course of patients with cutaneous polyarteritis nodosa 5. Cogan syndrome a. Clinical features 1. Recognize the clinical manifestations of Cogan syndrome 2. Understand the relationship of Cogan syndrome to other autoimmune diseases manifesting as hearing loss b. Treatment 1. Plan the medical management of a patient with Cogan syndrome c. Outcome 1. Recognize the complications of Cogan syndrome C. Leukocytoclastic Vasculitis 1. Henoch-Schoenlein purpura a. Definition 1. Know the definition of the clinical syndrome of Henoch-Schoenlein purpura b. Epidemiology 1. Recognize the epidemiologic features of Henoch-Schoenlein purpura, including age, gender, seasonal variation, relation to antecedent illness c. Pathogenesis 1. Understand the current concepts of the etiology and pathogenesis of Henoch-Schoenlein purpura d. Clinical features 1. Recognize the characteristic clinical manifestations of Henoch-Schoenlein

purpura 2. Identify the characteristic rash associated with Henoch- Schoenlein purpura 3. Identify the pattern and character of arthritis associated with Henoch-Schoenlein purpura Source: http://www.doksinet 4. Identify the gastrointestinal manifestations associated with Henoch-Schoenlein purpura 5. Recognize that characteristic testicular manifestations of Henoch-Schoenlein purpura 6. Identify the renal manifestations of Henoch-Schoenlein purpura 7. Identify the pulmonary manifestations of Henoch-Schoenlein purpura 8. Recognize the cardiac manifestations of Henoch-Schoenlein purpura 9. Recognize the CNS manifestations of Henoch-Schoenlein purpura e. Diagnosis 1. Know the classification criteria for the diagnosis of Henoch- Schoenlein purpura 2. Know the differential diagnosis in patients presenting with purpura and multisystem disease f. Pathology 1. Recognize renal pathology associated with Henoch-Schoenlein purpura 2. Recognize the histopathologic findings of Henoch-Schoenlein

purpura g. Laboratory findings 1. Interpret the common laboratory abnormalities in Henoch-Schoenlein purpura h. Radiologic changes 1. Recognize the characteristic radiographic abnormalities of Henoch-Schoenlein purpura i. Management 1. Know the medical management of Henoch-Schoenlein purpura 2. Understand the efficacy studies of glucocorticoids in HSP 3. Understand the efficacy studies of cyclophosphamide in children with HSP 4. Know the medical management of severe Henoch-Schoenlein purpura 5. Know the medical management of chronic Henoch-Schoenlein purpura 6. Understand the efficacy studies of plasmapheresis in children with Henoch-Schoenlein purpura j. Disease course and prognosis 1. Know the potential complications of Henoch-Schoenlein purpura 2. Hypersensitivity vasculitis and serum sickness a. Etiology 1. Know the possible inciting factors of serum sickness b. Clinical features 1. Know the clinical manifestations of serum sickness, including chronology and usual duration of each

c. Laboratory findings 1. Recognize the role of circulating immune complexes in serum sickness 2. Identify the characteristic laboratory abnormalities of serum sickness d. Pathology 1. Understand the histopathologic mechanisms of serum sickness e. Management 1. Plan the medical management of a patient with serum sickness 3. Hypocomplementemic urticarial vasculitis a. Clinical features Source: http://www.doksinet 1. Recognize the clinical manifestations of hypocomplementemic urticarial vasculitis 2. Recognize organ system involvement in a patient with hypocomplementic urticarial vasculitis 3. Know the usual time course of hypocomplementic urticarial vasculitis b. Laboratory findings 1. Interpret laboratory abnormalities in a patient with hypocomplementic urticarial vasculitis 4. Vasculitis associated with mixed cryoglobulinemia a. Etiology 1. Understand the role of hepatitis C virus in the pathogenesis and clinical manifestations of mixed cryoglobulinemia b. Clinical features 1.

Recognize the clinical manifestations of cryoglobulinemic vasculitis c. Laboratory findings 1. Interpret the characteristic laboratory findings of cryoglobulinemic vasculitis d. Management 1. Plan the management of a patient with cryoglobulinemic vasculitis D. Granulomatous Vasculitis 1. Allergic granulomatosis (Churg-Strauss) a. Etiology 1. Recognize the characteristics and clinical manifestations of allergic granulomatosis (Churg-Strauss) b. Laboratory findings 1. Identify the characteristic histopathologic findings in a patient with allergic granulomatosis (Churg-Strauss) 2. Identify the characteristic laboratory and radiographic findings in a patient with allergic granulomatosis (Churg Strauss) c. Management 1. Plan the medical management of a patient with allergic granulomatosis (Churg-Strauss) 2. Granulomatosis with polyangiitis (Wegener granulomatosis) a. Definition and classification 1. Know the classification criteria for granulomatosis with polyangiitis (Wegener

granulomatosis) 2. Understand the concept of limited granulomatosis with polyangiitis (Wegener granulomatosis) b. Clinical manifestations 1. Recognize the clinical features commonly associated with granulomatosis with polyangiitis (Wegener granulomatosis) 2. Know the differences between granulomatosis with polyangiitis (Wegener granulomatosis) in adults and children c. Differential diagnosis 1. Plan an appropriate diagnostic evaluation of a patient with respiratory and renal disease Source: http://www.doksinet 2. Know the differential diagnosis of granulomatosis with polyangiitis (Wegener granulomatosis), including other pulmonary or renal diseases, such as Goodpasture syndrome and microscopic polyangiitis 3. Recognize differences in clinical presentation, epidemiology, laboratory findings, and pathology between Goodpasture syndrome and granulomatosis with polyangiitis (Wegener granulomatosis) d. Pathology 1. Recognize the characteristic histopathologic abnormalities in a patient

with granulomatosis with polyangiitis (Wegener granulomatosis) e. Laboratory examinations 1. Interpret the characteristic laboratory findings in a patient with granulomatosis with polyangiitis (Wegener granulomatosis) 2. Know how to interpret ANCA results in granulomatosis with polyangiitis (Wegener granulomatosis) f. Radiologic changes 1. Interpret the characteristic radiologic abnormalities associated with granulomatosis with polyangiitis (Wegener granulomatosis) g. Treatment 1. Understand the appropriate use and efficacy of cyclophosphamide in children and adults with granulomatosis with polyangiitis (Wegener granulomatosis) 2. Plan the medical treatment of a patient with granulomatosis with polyangiitis (Wegener granulomatosis) 3. Understand the effectiveness studies of methotrexate in children and adults with granulomatosis with polyangiitis (Wegener granulomatosis) and other vasculitides h. Disease course and prognosis 1. Know the outcome of granulomatosis with polyangiitis

(Wegener granulomatosis) 2. Know the complications of granulomatosis with polyangiitis (Wegener granulomatosis), especially those affecting the hearing, vision, and airway 3. Primary angiitis of the central nervous system a. Clinical features 1. Recognize the clinical features commonly associated with primary angiitis of the central nervous system 2. Understand the prognosis of the different forms of isolated angiitis of the CNS b. Pathology 1. Recognize the characteristic histopathologic findings associated with primary angiitis of the central nervous system c. Diagnosis 1. Plan an appropriate diagnostic evaluation for a patient suspected as having primary angiitis of the central nervous system 2. Understand the relationship of vasoconstrictive agents to angiographic evidence of central nervous system vasculitis (eg, sympathomimetic agents, cocaine) 3. Plan the medical management of a patient with primary angiitis of the central nervous system E. Takayasu arteritis Source:

http://www.doksinet 1. Definition and classifications a. Know the classification of Takayasu arteritis according to distribution of affected vessels 2. Epidemiology a. Recognize the epidemiologic features of Takayasu arteritis 3. Etiology and pathogenesis a. Know the association of certain infections with Takayasu arteries in some parts of the world 4. Clinical manifestations a. Recognize the characteristic clinical features of Takayasu arteritis 5. Diagnosis a. Understand the value of different diagnostic techniques in a patient with Takayasu arteritis 6. Pathology a. Recognize the characteristic histopathologic findings and sites in Takayasu arteritis 7. Laboratory examinations a. Interpret the laboratory findings characteristic of Takayasu arteritis 8. Treatment a. Plan the medical management of a patient with Takayasu arteritis b. Understand the efficacy studies of angiotensin-converting enzyme inhibitors and angiotensin II receptor blocking drugs in hypertensive crisis 9.

Disease course and prognosis a. Know the outcome of Takayasu arteritis F. Giant Cell Arteritis 1. Know the clinical features, diagnostic modalities, and management of a patient with giant cell arteritis G. Other Vasculitic Syndromes 1. Behçet syndrome a. Definition and classification 1. Know the diagnostic criteria of Behçet syndrome b. Epidemiology 1. Recognize the epidemiologic features of Behçet syndrome (eg, age, geographic differences) c. Etiology and pathogenesis 1. Understand the etiology and pathogenesis of Behçet syndrome d. Clinical manifestations 1. General a. Recognize the clinical manifestations of Behçet syndrome 2. Mucosal disease a. Recognize the characteristic mucosal ulceration in patients with Behçet syndrome 3. Central nervous system and ocular disease a. Recognize the central nervous system and ocular manifestations of Behçet syndrome b. Recognize the ocular manifestations of Behçet syndrome 4. Arthritis Source: http://www.doksinet a. Recognize the

musculoskeletal manifestations of Behçet syndrome 5. Cutaneous a. Recognize the cutaneous manifestations of Behçet syndrome e. Diagnosis 1. Plan an appropriate diagnostic evaluation for a patient in whom Behçet syndrome is suspected f. Pathology 1. Know the histopathologic findings in Behçet syndrome g. Laboratory examinations 1. Recognize the characteristic laboratory abnormalities associated with Behçet syndrome, including immunogenetic markers h. Treatment 1. Plan the medical management of a patient with Behçet syndrome 2. Know the role of cell adhesion molecules 3. Understand the efficacy studies of glucocorticoids in Behçet syndrome i. Disease course and prognosis 1. Know the outcome of a patient with Behçet syndrome 2. Mucha-Habermann syndrome (also known as PLEVA) a. Know the clinical features, diagnostic modalities, and management of Mucha-Habermann syndrome 3. Sweet syndrome a. Know the pathology and clinical features of Sweet syndrome 4. Mimics of vasculitis a. Atrial

myxoma 1. Know the clinical features, diagnostic modalities, and management of atrial myxoma b. Infections 1. Identify rashes caused by infectious diseases (eg, Rocky Mountain spotted fever, meningococcemia) that resemble idiopathic vasculitis X. The Sclerodermas and Related Disorders A. General Considerations and Classifications 1. Classification a. Understand the classification of scleroderma 2. Epidemiology a. Recognize the difference in frequency of systemic and localized forms of scleroderma in children vs adults b. Be aware of the prevalence of scleroderma relative to other pediatric musculoskeletal diseases 3. Genetic background a. Be aware of any genetic associations in systemic sclerosis 4. Etiology a. Understand that one of the major defects in scleroderma is uncontrolled stimulation of cutaneous fibroblastic activity b. Know the current concepts of etiologic factors in scleroderma syndromes, including autoimmunity, chemical agents, infection, fetal-maternal "cell

transfusion" (microchimerism) Source: http://www.doksinet B. Localized Morphea (Scleroderma) 1. Definition and classification a. Morphea 1. Recognize the clinical manifestations of morphea, including plaque, generalized, and deep b. Linear morphea 1. Recognize the clinical manifestations of linear morphea, including scleroderma en coup de sabre, Parry-Romberg syndrome 2. Epidemiology a. Know the epidemiology of localized morphea, including frequency of different variants, gender ratio, and possible role of antecedent events 3. Laboratory examinations a. Know the laboratory findings in localized scleroderma b. Know the histopathology of the different forms of localized scleroderma 4. Differential diagnosis a. Be aware of other conditions that may mimic localized scleroderma clinically 5. Management of localized scleroderma a. Know the role of medical and surgical management of localized scleroderma b. Know the role of physical therapy in the management of localized scleroderma

c. Understand the effectiveness studies of methotrexate in children with linear scleroderma 6. Disease course and prognosis a. Know the natural history of localized scleroderma complications and the relationship to systemic disease C. Systemic Sclerosis 1. With diffuse skin thickening a. Clinical manifestations 1. Early signs and symptoms a. Recognize the general signs and symptoms of systemic sclerosis with diffuse skin thickening b. Know the common presenting manifestations of systemic sclerosis with diffuse skin thickening (eg, Raynaud phenomenon, hand stiffness) 2. Skin disease a. Recognize nailfold abnormalities and their correlations in systemic sclerosis with diffuse skin thickening b. Recognize the characteristic evolution of skin changes of systemic sclerosis, including edema, scleroderma, atrophy, telangiectasis, and calcinosis 3. Musculoskeletal disease a. Recognize the features, frequency, and significance of musculoskeletal involvement in systemic sclerosis with diffuse

skin thickening 4. Gastrointestinal involvement a. Recognize the features, frequency, and significance of esophageal and gastrointestinal involvement in systemic sclerosis with diffuse skin thickening 5. Cardiac involvement Source: http://www.doksinet a. b. c. d. e. Recognize the features, frequency, and significance of cardiac involvement in systemic sclerosis with diffuse skin thickening 6. Pulmonary disease a. Recognize the features, frequency, and significance of pulmonary involvement in systemic sclerosis with diffuse skin thickening 7. Renal disease a. Recognize the features, frequency, and significance of renal involvement in systemic sclerosis with diffuse skin thickening b. Understand the association of steroid use and renal crisis in systemic sclerosis Pathology 1. Identify histopathologic changes of the skin, lungs, kidneys, and other organs in systemic sclerosis with diffuse skin thickening Laboratory examinations 1. Recognize the frequency and clinical significance

of various autoantibodies in systemic sclerosis with diffuse skin thickening 2. Understand and interpret studies of internal organ involvement (ie, renal, gastrointestinal, cardiac function, bronchoalveolar lavage) in patients with systemic sclerosis with diffuse skin thickening 3. Understand the studies necessary to diagnose pulmonary hypertension in systemic sclerosis with diffuse skin thickening Radiographic changes 1. Know the indications for and interpretation of imaging studies in systemic sclerosis with diffuse skin thickening (eg, plain radiographs, high resolution CT, ultrasonography, radionuclide studies, MRI, thermography) Management 1. General supportive measures a. Understand the role of general supportive measures including avoidance of cold, skin care, physical and occupational therapy, splinting and biofeedback in scleroderma 2. Specific therapy of the disease process a. Know the indications for the use of medications (eg, penicillamine, corticosteroids, cytotoxic

drugs, immunosuppressives, methotrexate, photopheresis, biologics) and their risks in the treatment of systemic sclerosis with diffuse skin thickening 3. Raynaud phenomenon a. Know appropriate management approaches for Raynaud phenomenon, including medications, dosages, and side effects of calcium channel blockers and their risks in the treatment of systemic sclerosis with diffuse skin thickening 4. Renal disease a. Know the principles of management of renal disease in patients with systemic sclerosis with diffuse skin thickening 5. Gastrointestinal disease a. Know the principles of management of gastrointestinal disease in patients with systemic sclerosis with diffuse skin thickening Source: http://www.doksinet 6. Pulmonary disease a. Know the principles of management of pulmonary disease in patients with systemic sclerosis b. Know current treatment strategies for pulmonary hypertension in systemic sclerosis f. Disease course and prognosis 1. Know the disease course and prognosis

of systemic sclerosis with diffuse skin thickening 2. Recognize the factors that affect the prognosis of patients with systemic sclerosis with diffuse skin thickening 2. With limited skin thickening a. Clinical manifestations 1. General a. Recognize the association of anti-centromere antibodies and systemic sclerosis with limited skin thickening b. Be aware of the clinical course and prognosis of systemic sclerosis with limited skin thickening c. Know the distinctive clinical features of systemic sclerosis with limited skin thickening 2. Musculoskeletal disease a. Recognize the features, frequency, and significance of musculoskeletal involvement in systemic sclerosis with limited skin thickening 3. Gastrointestinal involvement a. Recognize the features, frequency and significance of gastrointestinal involvement in systemic sclerosis with limited skin thickening 4. Cardiac involvement a. Recognize the features, frequency, and significance of cardiac involvement in systemic sclerosis

with limited skin thickening 5. Pulmonary involvement a. Recognize the features, frequency, and significance of pulmonary involvement in systemic sclerosis with limited skin thickening 6. Renal disease a. Recognize the features, frequency, and significance of renal involvement in systemic sclerosis with limited skin thickening b. Pathology 1. Identify histopathologic changes of the skin and other organs in systemic sclerosis c. Laboratory examinations 1. Recognize the frequency and clinical significance of various autoantibodies in systemic sclerosis with limited skin thickening 2. Understand and interpret studies of internal organ involvement (ie, pulmonary, gastrointestinal, bronchoalveolar lavage) in patients with systemic sclerosis with limited skin thickening d. Radiographic changes Source: http://www.doksinet 1. D. E. F. G. Know the indications for and interpretation of imaging studies in systemic sclerosis with limited skin thickening (eg, plain radiographs,

high-resolution CT, ultrasonography, radionuclide studies, MRI) e. Management 1. Specific therapy of the disease process a. Know the indications for the use of medications (eg, penicillamine, corticosteroids, cytotoxic drugs, immunosuppressives, methotrexate, photopheresis, biologics) and their risks in the treatment of systemic sclerosis with limited skin thickening b. Understand the disease course and prognosis of systemic sclerosis with limited skin thickening Eosinophilic Fasciitis 1. Definition and classification a. Know the definition of eosinophilic fasciitis 2. Epidemiology a. Be aware of the effect of gender, age, and antecedent events on the incidence of eosinophilic fasciitis 3. Clinical manifestations a. Recognize the clinical manifestations of eosinophilic fasciitis and related syndromes 4. Pathology a. Recognize the histopathologic features of eosinophilic fasciitis 5. Laboratory examinations a. Interpret the laboratory findings in eosinophilic fasciitis 6. Treatment a.

Plan appropriate treatment approaches to eosinophilic fasciitis 7. Disease course and prognosis a. Know the prognosis of eosinophilic fasciitis Chronic Graft-Versus-Host Disease 1. Recognize the similarities and differences between graft-versus-host disease and scleroderma 2. Understand the treatment options in graft-versus-host disease, including corticosteroids, immunoregulatory drugs, biologicals, and physical therapy Chemically-Induced Scleroderma-Like Disease 1. Know the chemical agents associated with scleroderma-like syndromes Pseudosclerodermas 1. Phenylketonuria a. Know the association of morphea-like lesions with phenylketonuria 2. Syndromes of premature aging a. Recognize the skin changes associated with syndromes of premature aging including progeria and Werner syndrome 3. Localized idiopathic fibrosis and retroperitoneal fibrosis a. Know the clinical features of the localized idiopathic fibroses including retroperitoneal fibrosis, congenital torticollis, the fibromatoses,

Dupuytren contracture, lipogranulomatosis subcutanea and scleromyxedema 4. Scleredema Source: http://www.doksinet a. Know the features of scleredema and how it can be differentiated from scleroderma 5. Porphyria cutanea tarda a. Recognize the skin changes of porphyria cutanea tarda XI. Miscellaneous Rheumatologic Diseases A. Antiphospholipid Antibody Syndrome 1. Know that infarcts from antiphospholipid antibody syndrome can mimic vasculitic lesions 2. Know the clinical manifestations of antiphospholipid antibody syndrome 3. Know the treatment of antiphospholipid antibody syndrome 4. Understand the clinical importance of antibodies to beta2-glycoprotein 1 in antiphospholipid antibody syndrome 5. Know the pathology of antibodies to beta2-glycoprotein 1 in antiphospholipid antibody syndrome 6. Understand the laboratory tests used to detect antiphospholipid antibodies B. Sjogren Syndrome 1. Understand the clinical spectrum of Sjogren syndrome 2. Understand the conditions commonly

associated with the secondary form of Sjogren syndrome 3. Understand the pathology of salivary gland in Sjogren syndrome 4. Plan the management of dry eyes and dry mouth in a patient with Sjogren syndrome 5. Understand the serologic abnormalities reported in Sjogren syndrome, including NMO antibodies 6. Understand the association between Sjogren syndrome and lymphoproliferative and immunodeficiency diseases 7. Be familiar with the diagnostic studies used to assess salivary and lacrimal glandular function in Sjogren syndrome 8. Understand the association between Sjogren syndrome and renal disease 9. Understand the association between Sjogren syndrome and central nervous system disease C. Mixed Connective Tissue Disease 1. Understand the clinical patterns of mixed connective tissue disease and overlap syndromes in childhood 2. Understand the serologic patterns and associations of mixed connective tissue disease and overlap syndromes in childhood 3. Know the major organ system involvement

of mixed connective tissue disease, especially in children 4. Understand the special risks of pulmonary manifestations in childhood mixed connective tissue disease 5. Plan the management of mixed connective tissue disease, including drugs and physical measures 6. Understand the disease course of childhood mixed connective tissue disease and how it differs from adult-onset disease 7. Be aware of the diagnostic criteria for mixed connective tissue disease 8. Be aware of the histopathologic features of mixed connective tissue disease D. Thrombotic Thrombocytopenic Purpura 1. Understand the hematologic characteristics of thrombotic thrombocytopenic purpura Source: http://www.doksinet 2. 3. Plan an appropriate treatment regimen for thrombotic thrombocytopenic purpura Differentiate between thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome 4. Understand the characteristic pathology of thrombotic thrombocytopenic purpura 5. Understand the congenital and acquired forms of

thrombotic thrombocytopenic purpura 6. Understand the etiology and pathophysiology of thrombotic thrombocytopenic purpura E. Sarcoidosis 1. Recognize the clinical characteristics of childhood sarcoidosis 2. Differentiate the features of childhood sarcoid arthritis from those of adult-onset sarcoid arthritis 3. Distinguish between the uveitis of sarcoidosis and JIA 4. Recognize the pathology of sarcoidosis 5. Plan an appropriate treatment of childhood sarcoidosis 6. Know that certain types of sarcoidosis have vasculitis as a clinical feature 7. Be able to plan the work-up for a child with suspected sarcoidosis 8. Understand the differences between childhood sarcoidosis and Blau syndrome F. Relapsing Polychondritis 1. Know the diagnostic criteria for relapsing polychondritis 2. Recognize the clinical characteristics of arthritis in relapsing polychondritis 3. Plan treatment strategy for relapsing polychondritis in children 4. Recognize the life-threatening complications of relapsing

polychondritis G. Stevens-Johnson Syndrome 1. Recognize the clinical manifestations of Stevens-Johnson syndrome 2. Recognize the etiologic factors of Stevens-Johnson syndrome 3. Interpret the histopathologic findings characteristic of Stevens-Johnson syndrome 4. Plan the medical treatment of a patient with Stevens-Johnson syndrome 5. Know the differences between Stevens-Johnson syndrome, toxic epidermal necrolysis, and staphylococcal scalded skin syndrome H. Plant Thorn Synovitis 1. Recognize the relationship between plant thorn synovitis and an infectious agent I. Panniculitis/Erythema Nodosa 1. Know the classification of panniculitis 2. Interpret the pathology of panniculitis 3. Know the clinical manifestations of panniculitis in children 4. Plan an appropriate diagnostic evaluation in a patient suspected of having panniculitis 5. Understand the treatment of panniculitis in children 6. Know the definition of erythema nodosa 7. Interpret the pathology of erythema nodosa 8. Know the

clinical manifestations of erythema nodosa in children 9. Plan an appropriate diagnostic evaluation in a patient suspected of having erythema nodosa 10. Understand the treatment of erythema nodosa in children J. Primary and Secondary Raynaud 1. Define the clinical constellation of secondary Raynaud 2. Discuss the difference between primary and secondary Raynaud Source: http://www.doksinet 3. Know the frequency and impact of secondary Raynaud in the various rheumatic diseases 4. Know the difference between sympathetic overactivity and structural vascular disease 5. Understand the relationship between secondary Raynaud and digital gangrene K. Goodpasture Syndrome 1. Formulate a differential diagnosis for a patient in whom Goodpasture syndrome is suspected 2. Recognize the characteristic clinical manifestations of Goodpasture syndrome 3. Interpret the laboratory findings and histopathologic features characteristic of Goodpasture syndrome 4. Plan the medical management of a patient

with Goodpasture syndrome L. Miscellaneous Rheumatic Diseases 1. Be aware of chronic recurrent multifocal osteomyelitis as a cause of multifocal musculoskeletal signs and symptoms 2. Know the clinical manifestations of chronic recurrent multifocal osteomyelitis 3. Be able to differentiate the treatment of chronic recurrent multifocal osteomyelitis from multifocal septic osteomyelitis 4. Recognize the microscopic picture of a benign rheumatoid nodule 5. Recognize the difference between chronic recurrent multifocal osteomyelitis and SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis syndrome) 6. Know the clinical manifestations of SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis syndrome) 7. Know the clinical and laboratory manifestations of Goldbloom Syndrome (periostitis, dysproteinemia, elevation of erythrocyte sedimentation rate) 8. Know the clinical manifestations of Castleman disease 9. Know the rheumatic disease associations with Castleman disease 10. Know the

management of the different forms of Castleman disease XII. Immunodeficiencies and the Rheumatic Diseases A. Immunodeficiency Disorders 1. Recognize the type of infections (surface vs systemic), nature of the pathogens, frequency of infections, and family history that are all important as indicators of possible immunodeficiency 2. Know that the types of recurrent infections and the microbes that cause them can predict which part of the host defense mechanism is defective 3. Recognize that failure to thrive may be an indication of immunodeficiency 4. Understand the development of normal immune system and its function 5. Recognize which normal values for immunologic laboratory tests vary with age 6. Understand the significance of lymphopenia in infancy B. Disorders of Innate Immunity Associated with Rheumatic Diseases 1. Disorders of phagocytes a. Chronic granulomatous disease 1. Understand what organisms cause most infections in patients with chronic granulomatous disease 2. Know what

tests are used to confirm the diagnosis of chronic granulomatous disease 3. Know the inheritance patterns of chronic granulomatous disease Source: http://www.doksinet 4. Understand that mothers of children with X-linked chronic granulomatous disease are at increased risk of lupus b. Chediak-Higashi syndrome c. Other disorders of phagocytes 1. Recognize that gamma interferon receptor mutations limit the ability of macrophages to kill intracellular mycobacteria and salmonella 2. Recognize that defects in macrophage activation can cause a diffuse histiocytic pathology 2. Rheumatic diseases associated with complement deficiencies a. Inheritance 1. Know the inheritance pattern of serum complement deficiencies b. C1 deficiency 1. Understand that the phenotype of patients with C1 deficiency is typically a very severe, early onset SLE c. C2 deficiency 1. Know that serum C2 deficiency is the most common complement deficiency and that the frequency of the heterozygous state in the population

is approximately 1% d. C4 deficiency 1. Understand the genetics of C4 deficiency e. Late complement component deficiencies 1. Know the association and appropriate evaluation of late complement component deficiency with disseminated Neisseria infections f. C1 esterase inhibitor deficiency 1. Know that hereditary angioedema is caused by C1 esterase inhibitor deficiency and that some patients with HANE have an SLE-like syndrome 2. Know the appropriate tests to establish the diagnosis of C1 esterase inhibitor deficiency C. Disorders of Adaptive Immunity 1. Deficiencies of T and B lymphocytes a. Know the clinical and laboratory features of severe combined immunodeficiency b. Select appropriate blood products for administration to a patient with a T-cell defect c. Know that X-linked severe combined immunodeficiency is characterized by impaired T-cell maturation and a greatly decreased number of mature T cells, but that the count of B cells is usually normal or increased d. Know that

adenosine deaminase deficiency is a cause for autosomal recessive severe combined immunodeficiency 2. Humoral immunodeficiencies a. Isolated IgA deficiency 1. Know that isolated IgA deficiency is more frequent in patients with autoimmune disease 2. Know that individuals with absent IgA are susceptible to severe transfusion reactions caused by anti-IgA antibodies b. IgG Deficiencies Source: http://www.doksinet 1. Know the major forms of IgG deficiency: X-linked agammaglobulinemia, common variable immunodeficiency, and hyper-IgM immunodeficiency 2. Know that septic arthritis may be a presenting feature of humoral immunodeficiency 3. Know that children with common variable immunodeficiency frequently have autoimmune syndromes: atrophic gastritis, pernicious anemia, autoimmune cytopenias (Evans syndrome), inflammatory bowel disease, lymphoid interstitial pneumonia, JRA, etc 4. Know the genetic cause of X-linked agammaglobulinemia 5. Know the clinical features of X-linked hyper-IgM

syndrome 6. Know that patients with humoral immunodeficiency may develop chronic arthritis caused by Ureaplasma or Mycoplasma 7. Know that boys with unrecognized X-linked agammaglobulinemia are susceptible to chronic enteroviral infections, including vaccine-strain poliomyelitis 3. Miscellaneous a. Understand the most common phenotype of autoimmune lymphoproliferative syndrome (ALPS) b. Understand the immunodeficiencies associated with hemophagocytic syndrome c. Understand the most common phenotype of immune deficiency polyendocrinopathy, X-linked syndrome (IPEX) d. Understand the many autoimmune processes that occur in patients with APECED XIII. Arthritis Related to Infection A. Classification and Definitions 1. Appreciate the difference between septic, post-infectious and reactive arthritis B. Transient Synovitis of the Hip 1. Know the clinical features of transient synovitis of the hip, including age and gender predominance 2. Know the laboratory findings of transient synovitis of

the hip 3. Know the imaging findings of transient synovitis of the hip 4. Know the appropriate management and prognosis of transient synovitis of the hip C. Arthritis Caused by Bacteria 1. Clinical characteristics a. Be aware of the importance of systemic signs in septic arthritis b. Be aware of which joints are most commonly infected in septic arthritis c. Be aware that multiple joints may be infected in septic arthritis d. Be aware of the clinical features of septic arthritis 2. Etiology and pathogenesis a. Be aware of the relative importance of different organisms in the etiology of septic arthritis of different types b. Understand the mechanisms by which organisms cause post- infectious arthritis 3. Diagnostic procedures a. Synovial fluid aspiration and analysis 1. Recognize the urgency of performing joint aspiration in the management of suspected septic arthritis 2. Be aware of the synovial fluid abnormalities in septic arthritis Source: http://www.doksinet b. Radiographic

evaluation 1. Be aware of the usefulness and limitations of imaging techniques in septic arthritis, including x-rays, radionuclide scan, ultrasonography, and CT and MRI scans 4. Treatment a. Be aware of the appropriate treatment of specific causes of septic arthritis b. Be aware of the importance of open drainage in the treatment of septic arthritis of the hip 5. Prognosis a. Be aware of the possible long-term functional disability as a complication of septic arthritis 6. Special cases a. Gonococcal arthritis 1. Be aware of the occurrence of gonococcal arthritis in the adolescent age group 2. Be aware of the peculiar clinical features (increased prevalence in girls, relationship to menstruation and pregnancy, vesiculopustular rash, sexual abuse in the pre-adolescent child) of septic arthritis 3. Understand techniques to detect Neisseria gonorrhoeae b. Tuberculous arthritis 1. Know the epidemiology and clinical features of tuberculous arthritis c. Brucellosis 1. Know the epidemiology

and clinical features of arthritis associated with brucellosis d. Arthritis in immunocompromised patients 1. Know the importance of septic arthritis in immunocompromised patients 7. Related disorders a. Diskitis 1. Be aware of the clinical features of diskitis 2. Know how to diagnose diskitis 3. Be able to manage a patient with diskitis b. Osteomyelitis 1. Be aware of the importance of osteomyelitis as a cause of systemic symptoms and localized musculoskeletal symptoms 2. Be aware of the clinical features of osteomyelitis 3. Be aware of the importance of specific identification of causative organisms of osteomyelitis 4. Be aware of the relative usefulness of different imaging techniques in the diagnosis of osteomyelitis 5. Be familiar with the appropriate management of osteomyelitis 8. Miscellaneous a. Be aware that bacterial overgrowth can be associated with arthritis D. Arthritis Caused by Viruses 1. Be aware of the role of rubella virus and rubella vaccine as causes of persistent

arthritis 2. Be aware of the spectrum of disease caused by parvovirus Source: http://www.doksinet 3. Be aware of the fact that arthritis may be associated with viral infections: varicella, herpes simplex, cytomegalovirus, hepatitis C, mumps, alpha virus, Epstein-Barr virus, etc. 4. Be aware of the clinical syndrome of hepatitis, arthritis, and rash associated with hepatitis B virus infection 5. Know the clinical characteristics of arthritis caused by parvovirus B19 6. Know how to diagnose parvovirus B19-related arthritis E. Arthritis Caused by Fungi 1. Be aware that fungal arthritis can occur but is rare in the pediatric population F. Arthritis Caused by Spirochetes 1. Be aware of the epidemiologic features of Lyme disease 2. Know the major clinical manifestations of Lyme disease and their usual temporal sequence 3. Be aware of the causative organism, vector, and sensitivity and specificity of diagnostic tests for arthritis caused by spirochetes 4. Be aware of the indications for

treatment of Lyme disease 5. Be aware of the dosage and duration of treatment of Lyme disease at various stages in the illness 6. Recognize the clinical manifestations of Treponema pallidum infection 7. Understand the transmission of spirochetes from ticks to mammals (time required, preventive measures, etc.) 8. Understand the humoral response to the different Borrelia burgdorferi antigens and the diagnostic tests to identify the antibodies 9. Know the mechanisms by which spirochetes may persist in infected hosts despite a protective immune response (antigenic variation, etc.) 10. Be aware of drugs used in penicillin-allergic patients for the treatment of Lyme arthritis G. Post-Infectious Arthritis 1. Be aware of the pathogenesis of arthritis associated with bacterial endocarditis 2. Be aware of the pathogenesis of arthritis associated with infected shunts 3. Be aware of the pathogenesis of arthritis associated with meningococcemia H. Streptococcal Arthritis 1. Acute rheumatic fever a.

Be aware of the importance of rheumatic fever as a cause of morbidity throughout the world b. Be aware of the diagnostic criteria for rheumatic fever c. Recognize the typical features of joint involvement in rheumatic fever d. Describe the clinical manifestations of the classical rash of acute rheumatic fever e. Be aware of the theories of cross-reactive antigens in the pathogenesis of acute rheumatic fever f. Be aware of the hypothesis of B-cell antigens in susceptibility to acute rheumatic fever g. Know which laboratory investigations are indicated in the diagnosis and management of patients with acute rheumatic fever h. Understand the principles of acute and prophylactic treatment of acute rheumatic fever Source: http://www.doksinet i. j. k. Know the prognostic features of acute rheumatic fever Know the role of NSAIDs in the treatment of arthritis of rheumatic fever Know the specifics of endocarditis prophylaxis for patients with rheumatic heart disease l. Be aware of the

clinical features and the prognosis of chorea associated with rheumatic fever m. Define chorea in terms of frequency, relationship to cardiac disease, response to therapy, course of disease, and prognosis in rheumatic fever n. Know that acute rheumatic fever is caused by antibody to streptococcal cell wall antigen that crossreacts with myocardial antigen o. Know the exceptions to the Jones Criteria to establish a diagnosis of rheumatic fever p. Be aware of drugs used in penicillin-allergic patients for prophylactic treatment of acute rheumatic fever q. Know that chorea disappears with sleep r. Differentiate the clinical features of chorea caused by SLE and rheumatic fever 2. Post-streptococcal arthritis a. Know the characteristic features of post-streptococcal arthritis syndrome b. Know the treatment options for post-streptococcal arthritis I. Arthritis Associated with HIV 1. Be aware of the musculoskeletal manifestations of acute and chronic HIV infection XIV. Skeletal Malignancies

and Related Disorders A. Primary Tumors of Bone 1. Clinical manifestations a. Be familiar with the usual demographics of tumors of bone 2. Diagnostic procedures 3. Specific tumors of importance in childhood a. Benign tumors 1. Osteoid osteoma a. Recognize the clinical and imaging features of osteoid osteoma 2. Osteoblastoma a. Recognize the clinical and imaging features of osteoblastoma 3. Giant-cell tumor a. Recognize the clinical and imaging features of giant cell tumor 4. Osteochondroma a. Recognize the clinical and imaging features of osteochondroma 5. Tumor-like abnormalities of bone a. Langerhans cell histiocytosis 1. Recognize the clinical characteristics of histiocytosis syndromes b. Fibrous cortical defect 1. Recognize the clinical and imaging features of fibrous cortical defect c. Unicameral (solitary) bone cyst 1. Recognize the clinical and imaging features of a unicameral bone cyst d. Aneurysmal bone cyst 1. Recognize the clinical and imaging features of an aneurysmal bone

cyst b. Malignant tumors Source: http://www.doksinet 1. B. C. D. E. F. G. Osteosarcoma a. Recognize the clinical and imaging features of osteosarcoma 2. Ewing sarcoma a. Recognize the clinical and imaging features of Ewing sarcoma Malignancies that Metastasize to Bone 1. Recognize the clinical and imaging features of neuroblastoma 2. Recognize the clinical and imaging features of Wilms tumor 3. Plan appropriate laboratory tests to aid in the diagnosis of neuroblastoma Tumors of Synovium 1. Benign tumors a. Pigmented villonodular synovitis 1. Recognize the clinical, histopathologic, and imaging characteristics of pigmented villonodular synovitis b. Vascular malformations 1. Recognize the characteristics of vascular malformations c. Synovial chondromatosis 1. Recognize the characteristics of chondromatosis 2. Malignant tumors a. Synovial cell sarcoma 1. Recognize the characteristics of synovial sarcoma Tumors of Soft Tissue 1. Benign soft tissue tumors a. Recognize the

characteristics of benign musculoskeletal soft tissue tumors (juvenile fibromatosis) 2. Malignant soft tissue tumors a. Rhabdomyosarcoma 1. Recognize the characteristics of rhabdomyosarcoma b. Chondrosarcoma 1. Recognize the characteristics of chondrosarcoma c. Fibrosarcoma 1. Recognize the characteristics of fibrosarcoma Leukemia/Lymphoma 1. Recognize the musculoskeletal manifestations of acute leukemia/lymphoma in childhood 2. Distinguish the clinical features of JIA from those of leukemia/lymphoma 3. Recognize changes suggestive of leukemia/lymphoma in the results of commonly ordered laboratory tests (eg, increased concentrations of LDH, uric acid) 4. Distinguish the hematologic features of JIA from leukemia/lymphoma 5. Distinguish the radiologic features of JIA from leukemia/lymphoma Premalignant Conditions or Conditions Resembling Malignancy 1. Acquired Hemophagocytic Lymphohistiocytosis a. Recognize the clinical and histopathologic features of acquired hemophagocytic

lymphohistiocytosis Secondary Effects of Malignancies 1. Secondary hypertrophic osteoarthropathy Source: http://www.doksinet a. Recognize the clinical and radiologic characteristics of hypertrophic osteoarthropathy 2. Paraneoplastic phenomena a. Recognize the paraneoplastic phenomena associated with the malignancies of childhood XV. Bone and Connective Tissue Disorders A. Dysplasias 1. Understand that genetic mutations in development of connective tissue components have structural consequences 2. Understand the various types of osteochondrodysplasias: achondroplasia, multiple epiphyseal dysplasia, spondyloepiphyseal dysplasia, short-limb dwarfism, pseudoachondroplasia 3. Recognize that limb pain is associated with metaphyseal and diaphyseal dysplasia syndromes (eg, Engelmann disease) 4. Recognize the clinical and imaging manifestations of progressive pseudorheumatoid chondrodysplasia B. Metabolic and Nutritional Bone Disease 1. Osteoporosis a. Understand the causes of osteoporosis

in children b. Understand the clinical features of idiopathic juvenile osteoporosis c. Know the strategies to prevent the development of osteoporosis d. Recognize the radiologic features of osteoporosis e. Understand currently available methods to measure bone density f. Know the management of osteoporosis 2. Rickets a. Know the various types of rickets b. Know the musculoskeletal manifestations of rickets 3. Pseudohypoparathyroidism a. Recognize the clinical features of pseudohypoparathyroidism b. Understand the laboratory features of pseudohypoparathyroidism 4. Scurvy a. Understand the association of bone pain and hemarthrosis with scurvy 5. Hypervitaminosis A a. Understand the clinical features of vitamin A toxicity b. Understand that fad diets may contain high doses of vitamin A 6. Hyperlipoproteinemia a. Understand the musculoskeletal manifestations of hyperlipoproteinemia (eg, type 2 and 4) 7. Gout a. Understand the causes and evaluation of hyperuricemia in children b. Understand

the causes of gout in children c. Understand the management of gout in children 8. Ochronosis (alkaptonuria) a. Recognize the clinical features of ochronosis (alkaptonuria) 9. Hemoglobinopathies Source: http://www.doksinet a. Understand the musculoskeletal manifestations of sickle cell disease and other hemoglobinopathies, including hgb SC and hgb AS b. Differentiate between avascular necrosis and osteomyelitis in a patient with sickle cell disease 10. Kashin-Beck (Urov) disease a. Understand the musculoskeletal manifestation of Kashin-Beck disease b. Understand the current theories of pathogenesis of Kashin-Beck disease 11. Mseleni disease a. Know the clinical features of Mseleni disease b. Understand the current theories of pathogenesis of Mseleni disease C. Storage Diseases 1. Mucopolysaccharidoses a. Differentiate between Morquio disease and JIA b. Understand the clinical features of Scheie syndrome 2. Mucolipidoses a. Understand the clinical features of mucolipidoses 3.

Sphingolipidoses a. Understand the clinical and laboratory manifestations of the sphingolipidoses 4. Glycogen storage diseases a. Understand the clinical and laboratory features of glycogen storage disease D. Heritable Disorders of Connective Tissues 1. Osteogenesis imperfecta syndromes a. Know the biochemical defect in osteogenesis imperfecta b. Know the clinical varieties of osteogenesis imperfecta c. Be aware of the treatment strategies for osteogenesis imperfecta 2. Hypermobility syndrome a. Generalized hypermobility 1. Know that there are variations in hyperextensibility associated with embryologic development 2. Recognize the clinical presentation of generalized hypermobility b. Hypermobility syndrome 1. Be aware of the association of a positive family history in a child with hypermobility syndrome 2. Know the prognosis of hypermobility syndromes 3. Understand the relationship of hypermobility syndromes to other diseases of the connective tissues, specifically Ehlers-Danlos and

Marfan syndromes 4. Know the treatment of hypermobility syndromes 5. Be aware of the clinical criteria for the diagnosis and be able to plan the management of children with hypermobility syndromes 6. Identify the most likely complications of hypermobility syndromes c. Other hypermobility syndromes 1. Understand the inherited defects in Ehlers-Danlos syndrome 2. Understand the inherited defect in Marfan syndrome 3. Differentiate the clinical features of Marfan syndrome from those associated with disorders that can be confused with Marfan syndrome (eg, homocystinuria) Source: http://www.doksinet 3. Soft tissue calcification a. Understand the inherited defect and clinical characteristics of fibrodysplasia ossificans progressiva b. Understand the clinical features and causes of tumoral calcinosis 4. Trichorhinophalangeal syndrome a. Understand the musculoskeletal manifestations of trichorhinophalangeal syndrome 5. Nail-patella syndrome a. Understand that vasculitis and renal disease

can occur in nail-patella syndrome b. Be aware of the clinical features and the management of camptodactyly arthropathy coxavara pericarditis syndrome (OMIN208250) E. Auto-Inflammatory Disorders 1. Tumor necrosis factor receptor (TRAPS) a. Know the clinical and laboratory features and management of the tumor necrosis factor receptor disorders 2. MEFV abnormalities (FMF) a. Understand the efficacy studies of colchicine in the treatment of familial Mediterranean fever b. Understand that colchicine is associated with a decreased incidence of subsequent amyloidosis in familial Mediterranean fever c. Know the clinical and laboratory features and management of the MEFV disorders 3. CIAS abnormalities (NOMID) a. Understand the clinical features of neonatal-onset multisystem inflammatory disease b. Understand the current theories of pathogenesis of neonatal-onset multisystem inflammatory disease c. Recognize the radiographic features of neonatal-onset multisystem inflammatory disease d.

Recognize the role of the CIAS1 gene mutations in neonatal-onset multisystem inflammatory disease e. Know the clinical and laboratory management of the CIAS disorders 4. Nucleotide-binding oligomerization domain a. Know the clinical and laboratory features and management of the nucleotide-binding oligomerization domain 5. Mevalonic kinase abnormalities a. Know the clinical and laboratory features and management of the mevalonic kinase disorders 6. Periodic fever, aphthosis, pharyngitis, and adenitis syndrome a. Know the differential diagnosis of recurrent, persistent, or periodic fevers b. Recognize the genetic classification of periodic fever syndromes c. Understand the efficacy studies of glucocorticoids in PFAPA d. Know the clinical and laboratory features and management of the PFAFA disorders F. Miscellaneous Disorders 1. Hyperostoses a. Recognize the clinical characteristics of Caffey disease b. Identify the clinical conditions associated with various forms of hyperostosis c. Be

aware that retinoids cause hyperostosis and musculoskeletal syndromes Source: http://www.doksinet 2. Brachydactyly a. Identify the various clinical conditions associated with brachydactyly 3. Congenital soft tissue contracture a. Arthrogryposis 1. Recognize the clinical characteristics of arthrogryposis b. Camptodactyly syndromes 1. Understand the various syndromes associated with camptodactyly c. Fetal alcohol syndrome 1. Recognize the clinical features of fetal alcohol syndrome 4. Dupuytren contracture a. Identify a Dupuytren contracture, and manage appropriately 5. Acro-osteolysis syndromes a. Recognize syndromes associated with acro-osteolysis 6. Chondrolysis syndromes a. Understand the clinical and imaging characteristics of chondrolysis syndromes 7. Periostitis syndromes a. Formulate a differential diagnosis in a patient with suspected periostitis XVI. Musculoskeletal Manifestations of Other Chronic Diseases A. Diabetes Mellitus 1. Recognize the clinical features of diabetic

cheiroarthropathy 2. Know that insulin-resistant diabetes is caused by antibody to the insulin receptor B. Thyroid Disease 1. Understand the musculoskeletal manifestations of hypothyroidism and hyperthyroidism 2. Know that Grave disease is caused by antibody to TSH receptor C. Polyendocrinopathy 1. Understand the clinical and laboratory features of polyendocrinopathy D. Myasthenia Gravis 1. Understand the clinical features of myasthenia gravis 2. Know that myasthenia gravis is caused by antibody to acetylcholine receptor E. Cystic Fibrosis 1. Understand the features of two types of arthropathy associated with cystic fibrosis F. Hemophilia 1. Recognize acute hemarthrosis in a patient with hemophilia G. Hyperparathyroidism 1. Recognize the diagnosis of hyperparathyroidism based on skeletal manifestations and manage the clinical manifestations H. Whipple disease 1. Understand the role of Tropheryma whippelii in Whipple disease I. Celiac disease 1. Describe the arthritis that accompanies

celiac disease J. Erythema nodosum 1. Know the differential diagnosis and management of erythema nodosum K. Neutrophilic dermatoses 1. Pyoderma gangrenosum a. Know the clinical and laboratory characteristics of pyoderma gangrenosum Source: http://www.doksinet b. Know the appropriate treatment of pyoderma gangrenosum 2. Sweet syndrome a. Know the clinical and laboratory characteristics of sweet syndrome b. Know the appropriate treatment of Sweet syndrome L. Other 1. Know which rheumatic diseases have had immune complexes implicated in pathogenesis: RF-positive RA, acute rheumatic fever, SLE, and JRA 2. Know that pernicious anemia is caused by antibody to intrinsic factor of gastric parietal cells XVII. Rehabilitation A. Evaluation 1. Range of movement a. General 1. Recognize the common patterns of joint limitations and their functional implications in childhood arthritis b. Temporomandibular joint 1. Understand the relationship between chronic synovitis and the development of

deformity/destruction of the temporomandibular joints 2. Understand the orthodontic implications of temporomandibular joint disease and micrognathia c. Spine 1. Cervical a. Be familiar with the common problems involving the cervical spine in patients with JIA: fusion, subluxation, disk protrusion, impingement b. Recognize the potential risk and complications of anesthesia and surgery in a patient with JIA who has cervical spine involvement 2. Back a. Recognize the abnormalities of the axial skeleton in childhood rheumatic diseases: secondary scoliosis, kyphosis, lumbar lordosis, costovertebral involvement, sacroiliac/lumbosacral involvement b. Understand the consequences of abnormalities of the axial skeleton in childhood rheumatic diseases: secondary scoliosis, kyphosis, lumbar lordosis, costovertebral involvement, sacroiliac/lumbosacral involvement d. Upper extremities 1. Shoulder a. Recognize the common joint deformities and limitations in the shoulder: abduction (glenohumeral

range, scapular limitation), rotator cuff damage, internal/external rotation limitation b. Understand the mechanisms of shoulder deformities: abduction (glenohumeral range, scapular rotation), rotator cuff damage, internal/external rotation limitation c. Understand the functional implications of shoulder deformities: abduction (glenohumeral range, scapular rotation), rotator cuff damage, internal/external rotation limitation d. Understand the relationship between chronic synovitis and the development of deformity/destruction of the synovial joints Source: http://www.doksinet 2. Elbow a. Recognize the common elbow deformities: flexion contracture, flexion limitation, pronation and supination limitations b. Understand the mechanisms of elbow deformities: flexion contracture, flexion limitation, pronation and supination limitations c. Understand the functional implications of elbow deformities: flexion contracture, flexion limitation, pronation and supination limitations 3. Wrist a.

Recognize the common wrist deformities: decreased extension, subluxation, radial deviation, carpal tunnel syndrome, tendon rupture b. Understand the mechanism of wrist deformities: decreased extension, subluxation, radial deviation, carpal tunnel syndrome, tendon rupture c. Understand the functional implications of wrist deformities: decreased extension, subluxation, radial deviation, carpal tunnel syndrome, tendon rupture d. Understand the relationship between chronic synovitis and the development of deformity/destruction of the wrists 4. Hand a. Recognize the common hand deformities: thumb deformities, joint instability, radial and ulnar deviation, intrinsic tightness, buttoniere and swan neck deformities, tendon rupture, trigger fingers b. Understand the mechanisms of hand deformities: thumb deformities, joint instability, radial and ulnar deviation, intrinsic tightness, buttoniere and swan neck deformities, tendon rupture, trigger fingers c. Understand the functional implications

of hand deformities: thumb deformities, joint instability, radial and ulnar deviation, intrinsic tightness, buttoniere and swan neck deformities, tendon rupture, trigger fingers d. Understand the relationship between chronic synovitis and the development of deformity/destruction of the metacarpophalangeal, proximal interphalangeal and distal interphalangeal joints e. Lower extremities 1. Hip a. Recognize the mechanisms of common joint deformities: hip flexion contracture, hip rotation b. Understand the functional implications of common joint deformities: hip flexion contracture, hip rotation or abductor tightness c. Understand the soft tissue problems and their consequences associated with joint inflammation: abductor weakness, adductor spasm d. Understand the risk of subluxations related to ligament or muscle tightness/weakness around the hips e. Understand the relationship between chronic synovitis and the development of deformity/destruction of the hips 2. Knee a. Recognize the

mechanisms of common joint deformities: knee flexion contracture, knee valgus deformity, leg-length discrepancy Source: http://www.doksinet b. Understand the functional implications of common joint deformities: knee valgus deformity, leg-length discrepancy c. Recognize the soft tissue problems and their consequences associated with joint inflammation: quadriceps weakness, iliotibial band tightness d. Understand the risk of subluxations related to ligament or muscle tightness/weakness around the knees 3. Ankle/foot a. Understand the mechanism of common joint deformities: ankle plantar or dorsiflexion limitation, hindfoot valgus, decreased subtalar motion b. Understand the functional implications of common joint deformities: ankle plantar or dorsiflexion limitation, hindfoot valgus, decreased subtalar motion c. Recognize the soft tissue problems and their consequences associated with joint inflammation: tight heel cords, plantar fasciitis d. Recognize the risk of subluxations related

to ligament or muscle tightness/weakness around the metatarsals 2. Assessment of activities of daily living skills a. Understand how to assess age- and developmentally appropriate activities of daily living, including self-care such as dressing and eating, school and vocational activities b. Recognize how the results of functional status measures can influence rehabilitation of children with rheumatic diseases B. Treatment 1. Establishment of appropriate goals a. Be able to implement an appropriate rehabilitation plan and goals, including functional independence, maximum range and strength, ambulation, and school and vocational guidance 2. Specific treatment methods a. Positioning 1. Understand how to implement an appropriate positioning regimen in the management of children with rheumatic disease, to include techniques, risks, indications, and contraindications b. Stretching 1. Understand how to implement an appropriate stretching regimen in the management of children with rheumatic

disease, to include techniques, risks, indications, and contraindications c. Muscle strengthening (isometric, isotonic) 1. Understand how to implement an appropriate muscle strengthening (isometric, isotonic) regimen in the management of children with rheumatic disease, to includes techniques, risks, indications, and contraindications d. Splinting (resting, dynamic splints, and serial casting) 1. Recognize specific splinting methods applicable to the management of children with rheumatic disease, including techniques, risks, indications and contraindications of resting and dynamic splints e. Modalities (heat, cold, TENS, biofeedback) Source: http://www.doksinet 1. Understand the benefits, limitations and contraindications to the use of modalities applicable to the management of children with rheumatic diseases: heat, cold, ultrasound, transcutaneous nerve stimulation, functional electrical stimulation, biofeedback f. Shoe lifts 1. Be able to recommend appropriate shoe lifts to

correct leg-length discrepancies 2. Understand appropriate shoe modifications and orthoses to correct heel valgus/varus deformities and to relieve metatarsal pain g. Therapy after failure of intensive physical therapy 1. Understand the limitations of physical and occupational therapy and treatment options available after failure of intensive therapy h. Surgery 1. Recognize the indications for surgical intervention, including soft tissue releases, joint replacements, and soft tissue repair 2. Understand the expectations for surgical intervention, including soft tissue releases, joint replacements, and soft tissue repair 3. Be able to plan pre- and postoperative physical therapy management of a child with rheumatic disease C. Recognition of Clinical and Gait Analysis Patterns 1. Recognize the clinical and gait analysis patterns of a child with rheumatic disease and associated leg-length discrepancy 2. Understand the mechanism of gait abnormalities in a child with rheumatic disease and

associated leg-length discrepancy 3. Recognize the clinical and gait analysis patterns of a child with rheumatic disease and associated hip abductor weakness 4. Understand the mechanism of gait abnormalities in a child with rheumatic disease and associated hip abductor weakness 5. Understand the mechanism of gait abnormalities in a child with rheumatic disease and associated adduction contractures 6. Recognize the clinical and gait analysis patterns of a child with rheumatic disease and associated antalgic gait 7. Understand the mechanism of gait abnormalities in a child with rheumatic disease and associated antalgic gait 8. Understand the mechanism of gait abnormalities in a child with rheumatic disease and associated metatarsalgia 9. Understand the mechanism of Trendelenburg gait D. Management of Nonambulatory Patients 1. Understand the complications of a child with rheumatic disease becoming nonambulatory 2. Understand the contraindications to wheelchair use 3. Be able to plan the

rehabilitation of a nonambulatory patient E. Adaptive Equipment/Assistive Devices 1. Understand the biomechanics of the use of canes or crutches in unweighing a painful hip joint 2. Understand the principles of appropriate assistive devices for ambulation, including various types of canes, crutches, and walkers Source: http://www.doksinet 3. 4. Recognize the types of adaptive equipment that are available Understand the indications for and limitations of the use of adaptive equipment F. Supervision of Therapy Programs 1. Be able to prescribe an inpatient rehabilitation program, including indications, monitoring progress, and discharge planning 2. Be able to prescribe an outpatient rehabilitation program 3. Be able to prescribe a home rehabilitation program, including ability to train the parents XVIII. Psychosocial Issues/Developmental Issues/Chronic Illness A. Impact of Chronic Illness 1. Impact on child a. Know that a child with JIA can lose visual function and the resultant

ramifications b. Appreciate that malnutrition in a child with a chronic illness may be secondary to depression c. Appreciate that cosmetic effects of a chronic disease or its treatment affect the childs image d. Appreciate that poor self image secondary to chronic disease or its treatment may cause depression or psychosocial problems, such as acting out or poor compliance e. Differentiate between neuropsychiatric manifestations due to disease from those brought on by treatment or depression f. Recognize that psychosocial problems are an important cause of poor school achievement in chronically ill children g. Know that children with chronic rheumatic diseases will have an absentee rate from school above the national average h. Be aware of the academic potential of children with rheumatic diseases relative to their peers or siblings i. Understand the age-related differences in impact of JIA on psychosocial function j. Know the long-term psychosocial outcome, as well as the factors that

influence it, of children who had a rheumatic disease k. Appreciate that maternal depression may impact the outcome in children with rheumatic diseases 2. Impact on family a. Understand the physical constraints that chronic disease may impose on housing, transportation, and school b. Recognize that families of children with chronic illness will have substantial out-of-pocket (non-insured) expenses associated with obtaining medical and related services c. Understand the implications of a "pre-existing" illness on insurability d. Know how to help the family form reasonable expectations for a child with JIA e. Know that family members, including siblings and grandparents, often need support in dealing with a child with a chronic disability f. Recognize potential for increased parental conflict over the care of a child with a chronic illness g. Plan for the care of a family where there is conflict over the management of a child with a chronic illness B. Adherence (formerly called

Compliance) Source: http://www.doksinet 1. Know the factors that can contribute to poor adherence: dosing schedule, side effects, pill versus liquid, cost, age, psychosocial maturity, cosmetic effects C. Pain Management 1. Medication a. Be able to implement measures to facilitate adherence with a rehabilitation program b. Differentiate the pain-relieving qualities of anti- inflammatory drugs from those of pure analgesics c. Know the efficacy and side effects of commonly prescribed analgesics 2. Other modalities a. Know the principles of relaxation techniques for pain management b. Understand the role of heat and cold in pain management c. Understand the factors that can contribute to awareness of pain: age, fear, anxiety, fatigue, stress, inactivity d. Understand how pain, anxiety, and other behavioral factors affect cooperation with a rehabilitation program e. Understand the role of cognitive behavioral therapy in the management of pain f. Understand the role of acupuncture for

pain D. School 1. Physical education a. Know the possible solutions to the problems of participation in regular physical education classes: adaptive PE, swim therapy, alternative activities, no PE b. Understand the risks of high impact sports (football, wrestling, gymnastics) and their associated injury rates in children 2. Individualized educational plan a. Know the features of an Individualized Educational Plan b. Know the rights of the child and parents with regard to an Individualized Educational Plan c. Know the appeal process when an Individualized Educational Plan is rejected 3. Homebound education a. Understand the advantages and disadvantages of homebound education, including cyberschool b. Plan appropriate school reentry for a child receiving homebound education c. Be aware of the possible reasons for pediatric rheumatology patients requiring homebound teaching 4. School aids a. Know that modifications of physical environment at schools are possible for the handicapped child

b. Understand the various technical advancements that have contributed to the education of a physically handicapped child: tape recorders, computers, standing desks E. Special Issues in Adolescence 1. Effects on stages of sexual maturation and behavior a. Know the effects of severe disease and drug therapy on sexual maturation b. Understand that behavioral maturity should be sought even if sexual maturity is delayed in a chronically ill child Source: http://www.doksinet c. Be aware of the indications for endocrinologic consultation if sexual maturation is unduly delayed in a chronically ill child d. Understand the effects of short stature caused by disease or drug therapy on a chronically ill child 2. Compliance and independence a. Understand the difficulties with compliance that are unique to adolescent patients who are chronically ill b. Recognize the need for adolescents to develop independence while having a disease that fosters dependence c. Plan appropriate steps toward

independence for the adolescent with a rheumatic disease d. Plan appropriate steps to transition an adolescent with a rheumatic disease to adult health care providers 3. Sexual counseling and pregnancy a. Know the contraceptive methods available to adolescents with rheumatic diseases b. Know the risk of sexual transmission of AIDS and the principles of "safe sex" c. Know the effects of pregnancy on SLE and JIA and the effects of the diseases on the pregnancy d. Understand the need for sexual advice for adolescents with rheumatic diseases 4. Genetic counseling a. Know the risks of sibling recurrence of JIA b. Know the risks of transmission of SLE c. Know the risks of transmission of the spondyloarthropathies d. Know the risks to siblings of patients with JRA e. Know the risks to siblings of patients with SLE f. Know the risks to siblings of patients with ankylosing spondylitis g. Understand the possibility of guilt in families who have had rheumatic diseases in previous

generations 5. Vocational counseling a. Know the rights to and financial constraints of vocational counseling b. Be aware of age-appropriate vocational counseling for adolescents 6. Emotional problems a. Depression 1. Recognize the cardinal features of depression 2. Distinguish the degree of depression that requires professional consultation b. Suicide 1. Recognize the warning signs of suicide c. Dying 1. Know the indications for and the appropriate preparation of a chronically ill child and his or her family for the possibility of death F. Effect of Development Stage on Chronic Illness 1. Growth disturbance a. Know the manifestations of undergrowth or overgrowth of an extremity affected by a rheumatic disease b. Know that persistent muscle atrophy is possible in a child whose rheumatic disease occurred before 3 years of age Source: http://www.doksinet 2. Disease manifestations a. Understand that communicability of rheumatic disease to siblings or friends is a myth that must be

dispelled b. Understand various factors that may play a role in age-related differences in rheumatic diseases, including immaturity of the skeleton, reproductive organs, and immune system 3. Cognitive disturbances a. Recognize that poor school performance may be due to central nervous system involvement in patients with rheumatic disease b. Know the manifestations of organic brain syndrome and how to distinguish it from depression c. Recognize the association of neurodevelopmental delay in a young child with JIA 4. Understanding of illness, pain, etc. a. Understand the concept of illness behavior b. Know the long-term psychosocial outcome of children who had a rheumatic disease G. Legislative Issues 1. Know the specific content of PL 94-142 (Education for All Handicapped Children) 2. Know the specific content of section 504 of PL 93-112 3. Know the specific content of PL 94-482 4. Know the specific content of PL 99-457 5. Know the qualifications for patients with rheumatic disease to

qualify for Social Security disability benefits 6. Be familiar with characteristics of major third-party payor programs 7. Know the requirement for eligibility to services by SSA for a disabled child