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Updated: Jan 2 2023

Juvenile Idiopathic Arthritis

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  • summary
    • Juvenile Idiopathic Arthritis is a chronic autoimmune inflammatory arthritis lasting greater than 6 weeks in a patient younger than 16 years of age that typically presents with morning joint stiffness and ocular infections. 
    • Diagnosis is one of exclusion and presence of infection must be ruled out. One of the following must be present to confirm the diagnosis: rash, iridocyclitis, pericarditis, tenosynovitis, intermittent fever, presence of RF, C-spine involvement, morning stiffness. 
    • Treatment is usually immunomodulating drugs and frequent ophthalmologic exams.
  • Epidemiology
    • Demograpics
      • female > male
    • Anatomic location
      • joint involvement ( knee > hand/wrist > ankle > hip > C-spine)
  • Etiology
    • Genetics
      • HLA markers
        • DR4 associated with polyarticular
        • DR8, DR5, DR2.1 associated with pauciarticular
      • RF- seropositive in <15%
    • Diagnostic criteria
      • a diagnosis of exclusion
        • must rule out infection
      • one of the following must be present to make diagnosis
        • rash
        • presence of RF
        • iridocyclitis
        • C-spine involvement
        • pericarditis
        • tenosynovitis
        • intermittent fever
        • morning stiffness
    • Associated conditions
      • C-spine involvement
        • may lead to kyphosis, facet ankylosis, and atlantoaxial subluxation
      • Ocular involvement
        • typically consists of iridocyclitis, a type of anterior uveitis
        • frequently indolent and requires immediate ophthalmologic evaluation for slit lamp examination
        • can lead to rapid loss of vision if untreated
        • increase risk with positive ANA titer
      • Stills disease
        • acute-onset JRA with multiple joint involvement, fever, rash, and splenomegaly
        • infection must be ruled out
        • male = female
        • usually presents at age 5-10 years
  • Classification
    • Onset
      • early onset denotes onset before teens
      • late onset denotes onset during teens or later
      • Classification of Juvenile Rheumatoid Arthritis
      • Polyarticular (30%)
      • Hand/wrist involvement most common
      • Deformity is wrist ulnar deviation and flexion with MCP stiffness in extended, swollen & radially deviated digits position
      • 60% remission rate
      • > 5 joints involved
      • Small joint involvement (hand, wrist)
      • Symmetric findings
      •  F>M
      • Pauciarticular (50%)(oligoarticular)
      • Most common type
      • Early-onset associated with iridiocyclitis in 50% and chronic uveitis
      • Obtain ophthalmology consult; requires frequent ophthalmologic exams (q4 mth if ANA+; q6 mth if ANA-)
      • Girls are affected four times more often than boys in early-onset
      • Peak age 2-3 yrs
      • Late-onset seen more frequent in boys
      • Typical finding is a limp that improves during the day
      • Best prognosis for long term remission (70%)
      • < 5 joints involved
      • Large joint involvement (knees, ankles)
      • Asymmetric findings (leg length discrepancy, involved limb ends up LONGER)
      • M:F =1:4
      • Age 2-3y
      • Systemic (20%)
      • Includes Stills disease
      •  Poorest prognosis
      • systemic symptoms (rash, fever, multiple joint involvement) anemia, high WBC, elevated ESR, CRP, platelets
      • Hepatosplenomegaly, lymphadenopathy, pericarditis
      •  M=F, age 5-10y
  • Presentation
    • Symptoms
      • morning stiffness and joint pain
      • visual changes
      • fever
    • Physical exam
      • rash
      • iridocyclitis (can lead to rapid loss of vision if untreated)
  • Imaging
    • Radiographs
      • often negative at presentation
      • juxta-articular, late osteopenia and joint destruction can be seen if disease progressive
      • obtain flexion-extension neck radiographs to rule out atlantoaxial instability
  • Studies
    • Laboratory
      • rheumatoid factor
        • RF seropositive in <15%
          • associated with higher incidence of chronic, active, and progressive disease
          • often results in more destructive DJD
          • more likely to progress into adult RA
        • RF seronegative is more common
      • ANA
        • ANA positive is diagnostic
      • basic serology
        • values often normal and are not diagnostic
  • Treatment
    • Nonoperative
      • immunomodulating drugs (DMARDs) and frequent ophthalmologic exams
        • indications
          • first line of treatment
        • medications
          • DMARDs (disease modifying antirheumatic drugs)
            • new class of medications that have had significant impact on outcomes
            • includes but not limited to
              • etanercept
                • TNF inhibitor
              • rituximab
                • chimeric monoclonal antibody against CD20 on B cell surface
              • azathioprine
                • purine synthesis inhibitor
          • high dose aspirin/NSAIDs
            • salicylates are now used less frequently secondary to DMARD success
          • occasionally, gold may be used
          • intra-articular steroid injections
        • frequent ophthalmologic exams
          • slit-lamp examination twice yearly if ANA(-), every 4 months if ANA(+)
          • progressive iridocyclitis can lead to rapid loss of vision if untreated
    • Operative
      • synovectomy
      • epiphysiodesis
        • indications
          • LLD (affected leg typically longer in oligoarticular disease)
      • corrective osteotomies
        • indications
          • extremity deformity
          • deferred until skeletal maturitity
      • arthrodesis and arthroplasty
        • indications
          • for severe disease
  • Prognosis
    • 50% patients symptoms resolve without sequelae
    • 25% are slightly disabled
    • 25% have crippling arthritis or blindness
      • best prognosis pauciarticular > polyarticular > systemic
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