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.

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) &/or C-reactive protein (CRP), & the physician’s global assessment indicates no active disease
    • Patient on medication who have inactive disease for 6 consecutive months is considered to have as clinical remission on medication
    • Patients not taking any anti-arthritis or anti-uveitis medication & have inactive disease for 12 continuous months are considered to have clinical remission
  • Ten year remission rate in 50% of patients w/ oligoarthritis is noted; 40% in patients w/ systemic arthritis & 15% in patients w/ polyarthritis
    • Long-term remission in patients w/ rheumatoid factor (RF)-positive polyarthritis is unlikely
  • Follow up of patients depend on the disease severity & medications given; may be every 2 weesk-3 months
  • Activity of arthritis should be evaluated at least 3x/year & treatment be adjusted to reduce joint swelling & tenderness
  • Should identify complications of JIA such as macrophage activation syndrome (MAS)
    • An uncommon but potentially fatal complication of systemic-onset JIA
    • Patients are diagnosed through presence of ≥2 lab 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 CNS dysfunction, hemorrhages, or hepatomegaly) &/or lab criteria
    • Patient may also present w/ acute onset of severe anemia coupled w/ thrombocytopenia or leukopenia that have high spiking fevers, lymphadenopathy, & splenomegaly
    • May be differentiated from patients w/ exacerbation of systemic disease through fall of ESR secondary to hypofibrinoginemia & 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 JIA onset, w/ oligoarthritis subtype, w/ <4 years duration, w/ positive ANA & negative RF are considered at high risk to develop uveitis & should be screened every 3-4 months
    • Patients w/ medium risk (ie oligoarthritis or polyarthritis, ≥6 year old at onset, w/ negative ANA) should be screened every 6 months, & yearly for those at low risk (eg systemic-onset JIA)
  • Safety of treatment given should be regularly assessed
    • Monitoring for nonsteroidal anti-inflammatory drug (NSAID) use
      • Serum creatinine (Cr), complete blood count (CBC) & liver function test (LFT) should be measured prior to or soon after the initiation of treatment
      • Serum Cr, urinalysis, CBC & LFT should be checked 2x/year in patients maintained on long-term daily NSAIDs & yearly for patients taking NSAIDs routinely
    • Monitoring for Methotrexate use
      • Serum Cr, CBC & LFT should be measured prior to, 1 month after the initiation of treatment & 1-2 months after a dose escalation
      • 3-4 monthly measurement of serum Cr, CBC & LFT should be recommended in patients receiving stable dose of Methotrexate w/ 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 Cr, CBC & LFT should be tested prior to therapy & every 3-6 months thereafter
  • For patients who fail to have disease control w/ 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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