Juvenile Rheumatoid Arthritis

1. Juvenile Rheumatoid Arthritis Dr Althaf Ahmed 2005 MBBS

2. Objectives • recognize the characteristic symptoms and signs of the three clinical subgroups of JRA (JIA) • understand which laboratory and radiology studies are helpful when considering the diagnosis • be familiar with the differential diagnosis including infections, malignancies, and non-inflammatory conditions • understand the general approaches to treatment

3. JUVENILE RHEUMATOID ARTHRITIS • most frequent connective tissue disease of childhood • one of the more common chronic illnesses of childhood and an important cause of disability • currently defined in the U.S. by criteria established by ACR

4. JUVENILE RHEUMATOID ARTHRITISAmerican College of Rheumatology Revised Criteria • age of onset < 16 years • arthritis of one or more joints • duration of disease > 6 weeks • other conditions which present with arthritis in childhood must be excluded

5. JUVENILE RHEUMATOID ARTHRITISEpidemiology • described in all races and geographic areas • incidence: 6 – 19.6 cases/100,000 children • prevalence: 16-150/100,000 • females predominate 2:1

7. JUVENILE RHEUMATOID ARTHRITISEtiology and Pathogenesis • unknown • combination of factors • environment (infection, trauma, stress) • autoimmunity • immunogenetic

8. JUVENILE RHEUMATOID ARTHRITISOnset Types pauciarticular (< 4 joints) polyarticular (> 5 joints) systemic (arthritis with fever and rash)

9. ILAR Proposed Classification Criteria Juvenile Idiopathic Arthritis (JIA) systemic polyarticular RF+ polyarticular RF- oligoarticular persistent extended psoriatic arthritis enthesitis-related arthritis other arthritis

10. JUVENILE RHEUMATOID ARTHRITISClinical features: pauciarticular disease • 40-60% of patients with JRA • insidious onset • morning irritability/stiffness subtle • systemic symptoms: usually absent • large joints (rarely hip), asymmetric involvement • uveitis 20% • subtypes

12. JUVENILE RHEUMATOID ARTHRITISLaboratory Studies: Pauciarticular Disease • CBC: normal • ESR: usually normal • ANA: frequently positive • RF: usually negative • synovial fluid: class II (inflammatory) • x-ray findings: soft tissue swelling, periarticular osteoporosis, growth disturbance, loss of joint space

17. JUVENILE RHEUMATOID ARTHRITISClinical features: polyarticular disease • 30-40% of patients with JRA • morning irritability/stiffness more prominent • systemic symptoms: mild to moderate • large and small joints including cervical spine, symmetric involvement • uveitis 5% • subtypes

20. JUVENILE RHEUMATOID ARTHRITISLaboratory features: polyarticular disease • WBC , Hgb , platelets WNL to  • ESR  to   • ANA may be positive • RF may be positive • Synovial fluid: class II (inflammatory) • X-ray findings: soft tissue swelling, periarticular osteoporosis, joint space narrowing, erosions

22. JUVENILE RHEUMATOID ARTHRITISClinical features: systemic disease • 10-20% of patients with JRA • prominent systemic symptoms: fever, rash, lymphadenopathy, hepatosplenomegaly, pericarditis, pleuritis • arthritis may be absent for months to years • uveitis uncommon

26. JUVENILE RHEUMATOID ARTHRITISLaboratory studies: systemic disease • WBC   , Hgb , platelets  to  , • ESR   to    • ANA and RF usually negative • x-rays : soft tissue swelling

27. JUVENILE RHEUMATOID ARTHRITISExtra-articular Manifestations • generalized or local growth disturbances • delayed puberty • pericarditis, myocarditis, rarely endocarditis • plural effusion, rarely • pneumonitis, pulmonary fibrosis • hepatitis • hematuria

31. JUVENILE RHEUMATOID ARTHRITIS Differential Diagnosis

32. JUVENILE RHEUMATOID ARTHRITISTreatment • supportive not curative • involves multidisciplinary team approach • goals: • to suppress articular and/or systemic inflammation with as little risk as possible • to maintain function/prevent disabilities • to foster normal psychological and social development • heterogenity of disease mandates individualization

33. Pauci Articular • NSAID 2-4 weeks No response Change NSAID (2-4 weeks) No response Methotrexate no response Intra Articular Steroid

34. Poly Articular • NSAID (2-4 weeks) no response Change NSAID (2-4 weeks) no response 2 Methotrexate (10-15 mg/m/ week Steroid ( Bridge therapy) Newer drugs

35. Systemic Onset Less severe disease NSAIDs for 2 weeks. No response Change NSAIDs( 2 weeks) Oral steroids and taper slowly

36. Medications in the Treatment of JRA NSAID  intra-articular steroids sulfasalazine hydroxychloroquine (auranofin)  methotrexate (IM gold) (D-penicillamine)  etanercept azathioprine cyclophosphamide cyclosporin

37. JUVENILE RHEUMATOID ARTHRITISTreatment: physical measures heat: analgesia muscle relaxation splinting: provide joint rest maintain functional position correct deformities exercise: passive, active assisted and active range of motion general conditioning rest

38. JUVENILE RHEUMATOID ARTHRITISTreatment: education and supportive counseling • understand disease process, treatment and prognosis • understand roles in care • as normal possible: • discipline/family life • school • peer relationships • counseling

39. JUVENILE RHEUMATOID ARTHRITISPrognosis • chronic disease which cannot be cured • characterized by flares and remissions • after 10 years or more: • 31%-55% persistent active disease • 31% (9% - 48%) Steinbrocker Class III and IV

40. JUVENILE RHEUMATOID ARTHRITISPoor Prognostic Signs • pauciarticular • long duration of active disease • conversion to polyarticular disease (30%) • chronic uveitis • polyarticular • long duration of active disease • articular erosions • RF positivity/rheumatoid nodules • systemic • conversion to polyarticular disease (25-50%)

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