Updated: 1/2/2023

Juvenile Idiopathic Arthritis

Review Topic
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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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Flashcards (36)
1 of 36
Questions (8)

(OBQ18.52) A 5-year-old girl presents with an 8-week history of pain and swelling in the right knee, right shoulder, and left elbow after returning from summer camp. She denies any antecedent trauma, fevers, or rashes. Antibiotics prescribed by her primary care doctor have provided no significant relief, but she reports feeling better at the end of the day. Labs reveal a negative rheumatoid factor. Which of the following is most commonly associated with her diagnosis?

QID: 212948




Facial nerve palsy






Lower limb hemihypertrophy






L 2 A

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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

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



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



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



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



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



L 5 A

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

QID: 3252

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



Radiographic evaluation may be unremarkable



Cervical involvement may lead to atlantoaxial instability



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



Definitive diagnosis of JIA is confirmed by serologic evaluation



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

Before age 2 years



Between the ages of 2 and 4 years



Between the ages of 4 and 8 years



Between the ages of 8 and 12 years



During adolescence




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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

Skeletal survey



MRI of the pelvis



Clotting factor levels



Ophthalmology evaluation



Bone scan



L 2 D

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Evidence (8)
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