Rheumatology and Orthopedics· Topic 17.8 Systemic inflammatory and autoimmune syndromes· 17.8.10

Juvenile idiopathic arthritis

Juvenile idiopathic arthritis is arthritis beginning before age 16 and persisting at least 6 weeks with no other identifiable cause, spanning oligoarticular, polyarticular, systemic, enthesitis-related, and psoriatic subtypes. Diagnosis is clinical and antibody-subtyped; treatment starts with an NSAID and escalates through methotrexate to a biologic, while antinuclear-antibody-positive oligoarticular disease requires routine slit-lamp screening for silent chronic anterior uveitis.

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What this article covers

  • Overview

    Definition: juvenile idiopathic arthritis (JIA), formerly juvenile rheumatoid arthritis, is arthritis (joint swelling, or two of limited motion, tenderness, warmth) beginning before age 16 and…

  • Epidemiology

    Most common subtype: oligoarticular disease is the most frequent presentation, typically in young girls.

  • Risk factors

    Young female sex: the dominant demographic for oligoarticular and polyarticular disease.

  • Pathophysiology

    Chronic synovitis: immune-mediated inflammation of the synovium drives joint swelling, effusion, and progressive cartilage and bone injury if unchecked.

  • Clinical features

    Oligoarticular JIA: four or fewer joints, usually large and weight-bearing (knees, ankles); no systemic symptoms.

  • Differential diagnosis

    Acute lymphoblastic leukemia: leukocytosis, thrombocytosis, and lymphadenopathy can mimic systemic JIA, but marrow infiltration produces cytopenias and circulating blasts, not the cyclical fever and salmon rash of systemic disease.

  • Diagnosis

    The diagnosis is clinical: JIA is established by arthritis beginning before age 16 and persisting at least 6 weeks, with all other causes of childhood arthritis excluded; there is no single confirmatory test.

  • Management

    Start every subtype except systemic disease with an NSAID plus strengthening exercises: ibuprofen 5 to 10 mg/kg PO every 6 to 8 hours as needed, not exceeding 40 mg/kg/day, alongside physical and occupational therapy.

  • Complications

    Chronic anterior uveitis: silent, sight-threatening inflammation that can cause synechiae, cataract, glaucoma, and band keratopathy if screening is missed.

  • Prognosis

    Generally favorable: approximately 95% of JIA cases resolve by puberty; oligoarticular disease has the best long-term outlook when uveitis is caught early.

  • Prevention

    Schedule routine, not symptom-triggered, slit-lamp screening in every ANA-positive child, since chronic anterior uveitis in oligoarticular and ANA-positive polyarticular JIA is silent and otherwise goes undetected until damage occurs.

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