Arthritis, Juvenile Idiopathic
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- Juvenile idiopathic arthritis (JIA) is the most common chronic pediatric rheumatologic disease.
- JIA is associated with significant disability.
- Age of onset: <16 years
- Common symptoms: joint swelling, restricted range of motion, warmth, redness, pain
- Often ≥6 weeks of symptoms prior to diagnosis
- Seven (International League of Associations for Rheumatology [ILAR]) subtypes, determined by clinical characteristics in first 6 months of illness (1):
- Systemic (Still disease): 10%; preceded by febrile onset of ≥2 weeks with rash, serositis, hepatosplenomegaly or lymphadenopathy (1)
- Polyarticular rheumatoid factor (RF) (+): 2–7%; ≥5 joints involvement (1); large and small joints; RF positive on two tests ≥3 months apart (2)
- Polyarticular RF (−): 10–30%; ≥5 (large and small) joints involved (1); RF negative (2)
- Oligoarticular: 30–60%; involvement of 1 to 4 joints; risk for chronic uveitis in antinuclear antibodies (ANA) (+) females (1) and axial skeletal involvement in older boys (2). Types: (i) persistent (40%): knee, ankle, elbow; (ii) extended type (20%): >4 joints after first 6 months
- Psoriatic arthritis: 5%; arthritis with psoriasis or arthritis with >2 of: dactylitis, nail changes (pitting), psoriasis in first-degree relative (1)
- Enthesitis-related arthritis: 1–11%; arthritis and enthesis or one of them plus at least two of the following: sacroiliac or lumbosacral pain, Reiter syndrome or acute anterior uveitis in first-degree relative, acute symptomatic anterior uveitis, HLA-B27 (+), history of ankylosing spondylitis, sacroiliitis with inflammatory bowel disease, onset of arthritis in male >6 years old (1)[C]
- Undifferentiated arthritis (11–21%): presents with overlapping symptoms in ≥2 categories above or arthritis that does not fulfill above categories (2)
- System(s) affected: musculoskeletal, hematologic, lymphatic, immunologic, dermatologic, ophthalmologic, gastrointestinal
- Synonyms: juvenile chronic arthritis; juvenile arthritis; juvenile rheumatoid arthritis (JRA); Still disease (2)
- Male = female (1); onset: throughout childhood; 54% of cases occur in children 0 to 5 years.
- Polyarticular RF (+): female > male, 3:1 (2); onset: late childhood or adolescence (1)
- Polyarticular RF (−): female > male, 3:1; onset: early peak, 2 to 4 years; late peak, 6 to 12 years (2)
- Oligoarticular: female > male, 5:1; onset: 2 to 4 years (2)
- Psoriatic: female > male, 1: 0.95 (2); onset: early peak, 2 to 3 years; late peak, 10 to 12 years (1)
- Enthesitis: female > male, 1:7; onset: early peak, 2 to 4 years; late peak, 6 to 12 years (2)
- Affected patients have an increased risk of developing cancer, although short-term risk is low.
2 to 20/100,000 children <16 years in developed nations
16 to 150/100,000 children <16 years in developed nations (1)
Etiology and Pathophysiology
- Humoral and cellular immunodysregulation. T lymphocytes play a key role.
- Genetic predisposition; IL2RA/CD25 and VTCN1 implicated as genetic loci
- Environmental triggers, possibly infectious
- Immunoglobulin or complement deficiency
Female gender 3:1