Updated: 6/17/2021

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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Questions (8)
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(OBQ16.189) In which of the following clinical scenarios would an urgent ophthalmology consultation be warranted to mitigate potential irreversible complications of the primary pathology?

QID: 8951
1

A 4-year old male with proportionate dwarfism secondary to an autosomal recessive mutation resulting in L-alpha iduronidase deficiency.

9%

(127/1410)

2

A 5-year old male with proportionate dwarfism secondary to an X-linked recessive mutation resulting in sulpho-iduronate-sulphatase deficiency

11%

(155/1410)

3

A 6-year old female with 2 months of persistent left knee swelling and associated stiffness, intermittent fever, and elevated ESR.

38%

(542/1410)

4

A 7-year old male with developmental delay, dolichostenomelia, and positive urine nitroprusside test secondary to a cystathionine b-synthase deficiency.

18%

(248/1410)

5

A 10-year old developmentally normal male with dolichostenomelia, generalized ligamentous laxity, and pecrus carinatum.

23%

(323/1410)

L 5 A

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

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%

(289/2943)

2

Radiographic evaluation may be unremarkable

2%

(67/2943)

3

Cervical involvement may lead to atlantoaxial instability

8%

(227/2943)

4

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

5%

(140/2943)

5

Definitive diagnosis of JIA is confirmed by serologic evaluation

75%

(2215/2943)

L 2 C

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(SAE07PE.33) What is the peak period of onset in children with pauciarticular juvenile rheumatoid arthritis?

QID: 6093
1

Before age 2 years

0%

(0/180)

2

Between the ages of 2 and 4 years

19%

(34/180)

3

Between the ages of 4 and 8 years

38%

(68/180)

4

Between the ages of 8 and 12 years

32%

(58/180)

5

During adolescence

10%

(18/180)

N/A E

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(OBQ04.226) A 4-year-old female is brought by her parents in regard 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?

QID: 1331
1

Skeletal survey

5%

(93/1787)

2

MRI of the pelvis

4%

(70/1787)

3

Clotting factor levels

15%

(276/1787)

4

Ophthalmology evaluation

68%

(1209/1787)

5

Bone scan

8%

(135/1787)

L 2 D

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EXPERT COMMENTS (8)
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