Juvenile Rheumatoid Arthritis

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Juvenile Rheumatoid Arthritis

• Dorothy W. Wortmann, MD
• Clinical Associate Professor
• OU Department of Pediatrics

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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

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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

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JUVENILE RHEUMATOID ARTHRITISAmerican College of
Rheumatology Revised Criteria

• age of onset lt 16 years
• arthritis of one or more joints
• duration of disease gt 6 weeks
• other conditions which present with arthritis in
  childhood must be excluded

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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 21

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JUVENILE RHEUMATOID ARTHRITISEtiology and
Pathogenesis

• unknown
• combination of factors
• environment (infection, trauma, stress)
• autoimmunity
• immunogenetic

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JUVENILE RHEUMATOID ARTHRITISOnset Types

• pauciarticular (lt 4 joints)
• polyarticular (gt 5 joints)
• systemic (arthritis with fever and rash)

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ILAR Proposed Classification Criteria

• Juvenile Idiopathic Arthritis (JIA)
• systemic
• polyarticular RF
• polyarticular RF-
• oligoarticular
• persistent
• extended
• psoriatic arthritis
• enthesitis-related arthritis
• other arthritis

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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

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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

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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

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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

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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

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JUVENILE RHEUMATOID ARTHRITISLaboratory studies
systemic disease

• WBC ? ? , Hgb ?, platelets ? to ? ?,
• ESR ? ? to ? ? ?
• ANA and RF usually negative
• x-rays soft tissue swelling

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JUVENILE RHEUMATOID ARTHRITISExtra-articular
Manifestations

• generalized or local growth disturbances
• delayed puberty
• pericarditis, myocarditis, rarely endocarditis
• plural effusion, rarely
• pneumonitis, pulmonary fibrosis
• hepatitis
• hematuria

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JUVENILE RHEUMATOID ARTHRITIS

• Differential Diagnosis

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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

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Medications in the Treatment of JRA

• NSAID
• ?
• intra-articular steroids
• sulfasalazine
• hydroxychloroquine
• (auranofin)
• ?
• methotrexate
• (IM gold)
• (D-penicillamine)
• ?
• etanercept
• azathioprine
• cyclophosphamide
• cyclosporin

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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

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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

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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

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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)