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Juvenile Idiopathic Arthritis

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Topic updated on 04/22/16 6:37am
Introduction
  • A persistent autoimmune inflammatory arthritis lasting > 6 weeks in a patient younger than 16 years of age
    • previously known as juvenile rheumatoid arthritis
  • Epidemiology
    • joint involvement ( knee > hand/wrist > ankle > hip > C-spine)
    • female > male
  • 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
  • Prognosis
    • 50% patients symptoms resolve without sequelae
    • 25% are slightly disabled
    • 25% have crippling arthritis or blindness
      • best prognosis pauciarticular > polyarticular > systemic 
Classification
  • Onset
    • early onset denotes onset before teens
    • late onset denotes onset during teens or later

Classification of Juvenile Rheumatoid Arthritis
Polyarticular (30%)
  • > 5 joints involved
  • small joint involvement 
  • symmetric findings
  • hand/wrist involvement most common.
    • deformity is wrist ulnar deviation and flexion with MCP stiffness in extended, swollen & radially deviated digits position

Pauciarticular (50%)  (oligoarticular)


  • < 5 joints involved
  • large joint involvement
  • asymmetric findings


  • most common type
  • early-onset ssociated with iridiocyclitis in 50% and chronic uveitis. 
    • obtain opthalmology consult; requires frequent ophthalmologic exams
    • 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 day
Systemic (20%)
  • systemic symptoms
  • includes Stills disease
  • poorest prognosis

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
    • 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
    • corrective osteotomies
      • indications
        • extremity deformity
        • deferred until skeletal maturitity
    • arthrodesis and arthroplasty
      • indications
        • for severe disease

 

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Qbank (4 Questions)

TAG
(OBQ10.269) Which of the following statements is FALSE regarding juvenile idiopathic arthritis(JIA)? Topic Review Topic

1. To meet diagnostic criteria, persistent arthritis must occur in any joint for greater than 6 weeks before the age of 16 years
2. Radiographic evaluation may be unremarkable
3. Cervical involvement may lead to atlantoaxial instability
4. A patient with suspected JIA should undergo slit lamp examination by an ophthalmologist
5. Definitive diagnosis of JIA is confirmed by serologic evaluation

PREFERRED RESPONSE ▶
TAG
(OBQ04.226) A 4-year-old female is brought by her parents in regards to a right sided limp that improves during the day and has been present for two months. She is found to have a right knee effusion and associated soft-tissue swelling with no redness or warmth. Lab work reveals negative Rheumatoid factor, a positive low titer ANA and a normal WBC. Radiographs are normal for her age. What additional work up does she need? Topic Review Topic

1. Skeletal survey
2. MRI of the pelvis
3. Clotting factor levels
4. Ophthalmology evaluation
5. Bone scan

PREFERRED RESPONSE ▶




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