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Juvenile Rheumatoid Arthritis. Dr Althaf Ahmed 2005 MBBS. 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
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Juvenile Rheumatoid Arthritis Dr Althaf Ahmed 2005 MBBS
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
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
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
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
JUVENILE RHEUMATOID ARTHRITISEtiology and Pathogenesis • unknown • combination of factors • environment (infection, trauma, stress) • autoimmunity • immunogenetic
JUVENILE RHEUMATOID ARTHRITISOnset Types pauciarticular (< 4 joints) polyarticular (> 5 joints) systemic (arthritis with fever and rash)
ILAR Proposed Classification Criteria Juvenile Idiopathic Arthritis (JIA) systemic polyarticular RF+ polyarticular RF- oligoarticular persistent extended psoriatic arthritis enthesitis-related arthritis other arthritis
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
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
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
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
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
JUVENILE RHEUMATOID ARTHRITISLaboratory studies: systemic disease • WBC , Hgb , platelets to , • ESR to • ANA and RF usually negative • x-rays : soft tissue swelling
JUVENILE RHEUMATOID ARTHRITISExtra-articular Manifestations • generalized or local growth disturbances • delayed puberty • pericarditis, myocarditis, rarely endocarditis • plural effusion, rarely • pneumonitis, pulmonary fibrosis • hepatitis • hematuria
JUVENILE RHEUMATOID ARTHRITIS Differential Diagnosis
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
Pauci Articular • NSAID 2-4 weeks No response Change NSAID (2-4 weeks) No response Methotrexate no response Intra Articular Steroid
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
Systemic Onset Less severe disease NSAIDs for 2 weeks. No response Change NSAIDs( 2 weeks) Oral steroids and taper slowly
Medications in the Treatment of JRA NSAID intra-articular steroids sulfasalazine hydroxychloroquine (auranofin) methotrexate (IM gold) (D-penicillamine) etanercept azathioprine cyclophosphamide cyclosporin
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
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
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
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%)