Juvenile idiopathic arthritis was formerly known as juvenile rheumatoid arthritis or juvenile chronic arthritis.
It presents as chronic joint swelling, pain with functional limitation for at least 6 weeks of unknown cause that starts before 16 year of age.
It is the most common autoimmune-autoinflammatory disease in children.
Around half of the children with juvenile idiopathic arthritis may have active disease until adulthood.

Juvenile%20idiopathic%20arthritis Management

Follow Up

  • Patients are considered to have inactive disease if they have no active synovitis, fever, rash, serositis, splenomegaly, generalized lymphadenopathy, or active uveitis, have normal erythrocyte sedimentation rate (ESR) and/or C-reactive protein (CRP), and the physician’s global assessment indicates no active disease
    • Patient on medication who have inactive disease for 6 consecutive months is considered to have clinical remission on medication
    • Patients not taking any anti-arthritis or anti-uveitis medication and have inactive disease for 12 continuous months are considered to have clinical remission
  • Ten year remission rate in 50% of patients with oligoarthritis is noted; 40% in patients with systemic arthritis and 15% in patients with polyarthritis
    • Long-term remission in patients with rheumatoid factor (RF)-positive polyarthritis is unlikely
  • Follow up of patients depend on the disease severity and medications given; may be every 2 weeks-3 months
  • Activity of arthritis should be evaluated at least 3x/year and treatment be adjusted to reduce joint swelling and tenderness
  • Should identify complications of juvenile idiopathic arthritis such as macrophage activation syndrome (MAS)
    • An uncommon but potentially fatal complication of systemic-onset juvenile idiopathic arthritis
    • Patients are diagnosed through presence of ≥2 laboratory criteria (eg ≤262 x109/L platelet count, >59 U/L aspartate aminotransferase (AST), ≤4 x109/L white blood cell (WBC), or ≤2.5 g/L fibrin), or any ≥2 clinical (eg central nervous system dysfunction, hemorrhages, or hepatomegaly) and/or laboratory criteria
    • Patient may also present with acute onset of severe anemia coupled with thrombocytopenia or leukopenia that have high spiking fevers, lymphadenopathy, and splenomegaly
    • May be differentiated from patients with exacerbation of systemic disease through fall of ESR secondary to hypofibrinoginemia and hepatic dysfunction
    • Diagnosis is confirmed by hemophagocytosis seen in bone marrow biopsy
    • High-dose intravenous (IV) Methylprednisone, Cyclosporine, or Anakinra may be effective emergency treatment
  • Screening of comorbidities should also be done
  • TB testing should be repeated at least once every year in all patients maintained on anti-TNF-α agents
  • Patients who are female, <6 years old at juvenile idiopathic arthritis onset, with oligoarthritis subtype, with <4 years duration, with positive antinuclear antigen (ANA) and negative RF are considered at high risk to develop uveitis and should be screened every 3-4 months
    • Patients with medium risk (ie oligoarthritis or polyarthritis, ≥6 year old at onset, with negative ANA) should be screened every 6 months, and yearly for those at low risk (ie systemic-onset juvenile idiopathic arthritis)
  •  Screening for uveitis associated with juvenile idiopathic arthritis should be done regularly and treatment should be initiated based on current evidenced-based guidelines to prevent complications and to preserve vision
    • Screening should be done within 6 weeks in newly diagnosed patients with juvenile idiopathic arthritis
  • Safety of treatment given should be regularly assessed
    • Monitoring for nonsteroidal anti-inflammatory drug (NSAID) use
      • Serum creatinine, complete blood count (CBC) and liver function test (LFT) should be measured prior to or soon after the initiation of treatment
      • Serum creatinine, urinalysis, CBC and LFT should be checked 2x/year in patients maintained on long-term daily NSAIDs and yearly for patients taking NSAIDs routinely
    • Monitoring for Methotrexate use
      • Serum creatinine, CBC and LFT should be measured prior to, 1 month after the initiation of treatment and 1-2 months after a dose escalation
      • 3-4 monthly measurement of serum creatinine, CBC and LFT should be recommended in patients receiving stable dose of Methotrexate with no recent history of abnormal lab results
      • Lab tests should be done 1-2 days before the scheduled weekly dose of Methotrexate
      • For abnormal LFT results, the following are recommended:
        • LFT 2x upper limit of normal (ULN): No specific action or recheck LFT at shorter interval
        • LFT >2x ULN: Reduce dose or temporarily stop the medication
        • LFT >3x ULN even after decreasing the dose: Discontinue use
    • Monitoring for anti-TNF-α use
      • If drug will be used continuously, serum creatinine, CBC and LFT should be tested prior to therapy and every 3-6 months thereafter
  • For patients who fail to have disease control with any of the medications, autologous stem cell transplantation may be an option to achieve disease remission, but is still currently being evaluated
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