Updated: 7/24/2018

Juvenile Idiopathic Arthritis

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Snapshot
  • A 3-year-old boy presents to the emergency department due to worsening joint pain, fever, and a new rash. He is accompanied by his mother who reports that he has been pointing at his knee and hip while crying. She believes he has been having this symptom for approximately 2 months. He has been having 1-2 fevers a day, and she reports seeing a "pink-like" rash. On physical exam, there is an evanescent salmon-colored rash on the left thigh. There is tenderness to palpation of the left knee and hip with limited range of motion. Lab results show an elevated ESR, CRP, and positive ANA. A pediatric rheumatologist is consulted for further evaluation.
Introduction
  • Clinical definition
    • a broad term that encompasses different types of pediatric chronic arthritis that is characterized by joint pain and inflammation
      • 1 or more joints are involved for at least 6 weeks in patients < 16 years of age
      • subtypes
        • polyarticular juvenile idiopathic arthritis (JIA)
          • ≥ 5 joints involved
        • pauciarticular JIA
          • 1-4 joints involved
        • systemic JIA
          • visceral involvement
  • Epidemiology
    • incidence
      • the most common chronic rheumatologic disorder in children
    • demographics
      • more frequently with European ancestry
  • Pathogenesis
    • not well understood but may involve both genetic and environmental factors
      • the humoral and cell-mediated immune system are involved
        • T-cells release tumor necrosis factor-α (TNF-α), interleukin-6 (IL-6), and IL-1
        • the humoral immune system is involved by increasing antinuclear antibody (ANA) production, immunoglobulins, and circulating immune complexes
  • Prognosis
    • postitive
      • improved with advances in treatment
    • negative
      • early hip or wrist involvement
      • presence of rheumatoid factor
Presentation
  • Symptoms
    • fever
      • quotidian pattern
        • fevers occurring once or twice per day that eventually subside
      • morning stiffness and joint pain
        • joint stiffness and pain can also be noted with prolonged periods of inactivity (e.g., sitting)
      • visual changes
        • can be suggestive of a uveitis
  • Physical exam
    • joint involvement
      • swelling
      • limited range of motion
      • tenderness to palpation
    • uveitis
    • visceral involvement in cases of systemic JIA such as
      • evanescent and salmon-colored macular rash
      • hepatosplenomegaly
      • serositis
        • e.g., pleural and pericardial effusion
Imaging
  • Radiography
    • indication
      • performed if only 1 joint is involved in order to evaluate for other causes (e.g., osteomyelitis)
Studies
  • Labs
    • c-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)
      • typically in systemic and polyarticular JIA
    • positive ANA
      • in most cases
  • Diagnostic criteria
    • a patient < 16 years of age with 1 or more joints involved for at least 6 weeks
Differential
  • Reactive arthritis
  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Leukemia
  • Pediatric sarcoidosis
Treatment
  • Conservative
    • period ophthalmologic exams
      • indication
        • for monitoring of eye involvement (e.g., uveitis)
  • Medical
    • nonsteroidal antiinflammatory drugs (NSAIDs)
      • indication
        • for symptomatic relief
    • disease-modifying antirheumatic drugs (DMARD)
      • indication
        • for controlling inflammatory process
      • medication
        • methotrexate
        • etanercept
        • adalimumab
    • intra-articular glucocorticoids
      • indication
        • effective in managing inflamed joint
Complications
  • Infection
  • Macrophage-activation syndrome
  • Pericarditis
  • Hemolytic anemia
  • Enarteritis
 

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