Updated: 7/2/2017

Juvenile Idiopathic Arthritis

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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 (hand, wrist)
  • symmetric findings
  • F>M
  • 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

Pauciarticular (50%)  (oligoarticular)


  • < 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


  • most common type
  • early-onset ssociated with iridiocyclitis in 50% and chronic uveitis. 
    • obtain opthalmology consult; requires frequent ophthalmologic exams (q4mth if ANA+; q6mth 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 day
  • best prognosis for long term remission (70%)
Systemic (20%)
  • systemic symptoms (rash, fever, multiple joint involvement) 
  • anemia, high WBC, elevated ESR, CRP, platelets
  • hepatosplenomegaly, lymphadenopathy, pericarditis
  • M=F, age 5-10y
  • 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
    • 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
 

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Questions (5)
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(OBQ10.269) Which of the following statements is FALSE regarding juvenile idiopathic arthritis(JIA)? Review Topic

QID: 3252
1

To meet diagnostic criteria, persistent arthritis must occur in any joint for greater than 6 weeks before the age of 16 years

10%

(215/2175)

2

Radiographic evaluation may be unremarkable

2%

(53/2175)

3

Cervical involvement may lead to atlantoaxial instability

8%

(171/2175)

4

A patient with suspected JIA should undergo slit lamp examination by an ophthalmologist

5%

(99/2175)

5

Definitive diagnosis of JIA is confirmed by serologic evaluation

75%

(1634/2175)

ML 2

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PREFERRED RESPONSE 5
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(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? Review Topic

QID: 1331
1

Skeletal survey

5%

(74/1354)

2

MRI of the pelvis

4%

(48/1354)

3

Clotting factor levels

14%

(195/1354)

4

Ophthalmology evaluation

70%

(943/1354)

5

Bone scan

7%

(92/1354)

ML 2

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PREFERRED RESPONSE 4
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