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common rheumatologic diagnoses
Dustin Fleck, MD pediatric rheumatology
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pediatric rheumatology
autoimmune and autoinflammatory conditions can affect all organs primarily affects: connective tissues (muscle, joints, tendons)
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rheum clinic diagnoses
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juvenile idiopathic arthritis (JIA)
JIA v. JRA?
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JRA EULAR criteria (1977) pauciarticular polyarticular systemic
***JRA has been clinically used term since the 1940s
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JCA EULAR criteria (1978) pauciarticular polyarticular systemic
juvenile rheumatoid arthritis (JRA) juvenile psoriatic arthritis juvenile ankylosing arthritis
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JIA ILAR criteria (1997) oligoarticular systemic polyarticular
extended oligoarticular persistent systemic seronegative polyarticular JIA seropositive enthesis related arthritis psoriatic arthritis other
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arthritis defined as joint effusion alone limited range of motion
or (2 of 3) limited range of motion tenderness or pain on motion increased warmth of joint
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juvenile idiopathic arthritis (JIA)
>6 weeks <16 years of age exclude other diagnoses
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juvenile idiopathic arthritis
6 months to 16 years of age 1 in 250-1,000 children affected females > males (2:1)
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after 6 months: still less than 5
oligoarticular jia less than 5 joints after 6 months: still less than 5 after 6 months: 5 or more persistent oligo JIA extended oligo JIA
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polyarticular jia involving more than 4 joints in 1st 6 months
+ RF/CCP -RF/CCP seropositive poly JIA seronegative Poly JIA
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prognosis persistent oligo> seronegative poly > extended oligo > seropositive poly
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enthesitis related arthritis
ERA, spondyloarthropathy juvenile ankylosing apondylitis age: (males 4:1)
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enthesitis related arthritis
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juvenile ankylosing spondylitis
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juvenile ankylosing spondylitis
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juvenile psoriatic arthritis
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juvenile psoriatic arthritis
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juvenile psoriatic arthritis
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juvenile psoriatic arthritis
+ 2 of the following dactylitis nail pits 1st degree relative with psoriasis arthritis + psoriasis or
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making the diagnosis physical exam exclusion
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physical exam
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physical exam
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does lab work confirm the diagnosis of JIA?
the workup does lab work confirm the diagnosis of JIA?
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anti-nuclear antibody (ANA)
Figure 1: antibody binding of proliferating cell nuclear antigen (PCNA) in the systemic lupus erythematous serum. Image downloaded from Biomedical Central. Arthritis Research and Therapy. Tan, Eng. 12:104 (2014)
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anti-nuclear antibody (ANA)
30% of healthy children will have a positive ANA on laboratory screen
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anti-nuclear antibody (ANA)
9,782 children would have a positive ANA on laboratory screening in Dayton, Ohio
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anti-nuclear antibody (ANA)
Anti-DsDNA Anti-Sm Anti-SSA Anti-SSB Anti-Scl70 Anti-Jo Anti-RNP Anti Ribosomal Anti- Chromatin ANA
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what role does ANA play in JIA?
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uveitis
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uveitis Positive ANA + 6 years or younger = High risk
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rheumatoid factor
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rheumatoid factor tissue damage complement activation
inflammatory cytokines tissue damage
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rheumatoid factor positivity
Rheumatoid arthritis 80%
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Bacterial endocarditis
Healthy 70 year old Rheumatoid arthritis 80% Multiple Vaccines 15% Coxsackie B virus 15% Bacterial endocarditis 40% EBV and CMV infections 20%
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Bacterial endocarditis
jra 5% Healthy 70 year old Rheumatoid arthritis 80% Multiple Vaccines 15% Coxsackie B virus 15% Bacterial endocarditis 40% EBV and CMV infections 20%
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anti-cyclic citrullinated peptide antibody
antiperinuclear factor (1964) antikeratin antibodies (1979) anti-sa antibodies
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RF v. CCP sensitivity specificity RF -> 84.8% CCP -> 74%
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RF v. CCP RF -> extra-articular manifestation
CCP -> articular damage/erosion
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Treatment NSAIDS DMARDS Other Biologics TNF alpha inhibitors
RF + triple therapy NSAID methotrexate hydroxychloroquine NSAIDS Intra-articular corticosteroid injection DMARDS Ankylosing spondylitis TNF alpha inhibitors Other Biologics
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take home points JIA is a common chronic disease in children
JIA is a clinical diagnosis made off physical exam laboratory studies are for risk/sub-categorization
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