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Types of juvenile idiopathic arthritis

Authoring team

International League of Associations for Rheumatology (ILAR) has proposed the following criteria which classifies JIA into 7 heterogeneous subgroups

  • oligoarticular
    • no more than four affected joints in the first six months of disease
    • 27–56% of JIA cases
    • F>M
    • often antinuclear antibody (ANA) positive. Strong association with anterior uveitis
  • polyarticular – RF negative
    • more than four affected joints in the first six months of disease; RF negative
    • 11–28% of JIA cases
    • F>M
    • often symmetric involvement of small, medium and large joints
  • polyarticular – RF positive
    • more than four affected joints in the first six months of disease; RF positive on two separate occasions
    • 2–7% of JIA cases
    • F>M
    • closely related to rheumatoid arthritis with similar phenotypic and prognostic features; classically affects adolescent girls
  • enthesitis-related arthritis
    • arthritis, enthesitis – tends to involve axial and weight-bearing joints including SIJ
    • 3–11% of JIA cases
    • M>F
    • the juvenile spondyloparthropathy, often HLA-B27 positive males
  • psoriatic arthritis
    • arthritis, psoriasis
    • 2–11% of JIA cases
    • F>M
    • also associated with dactylitis and nail changes such as pitting, ridging, onycholysis
  • systemic onset
    • fever, rash, arthritis (‘Still’s disease’); may have hepatosplenomegaly, lymphadenopathy
    • 4–17% of JIA cases
    • F>M
    • can develop life-threatening immune dysregulation known as macrophage activation syndrome
  • undifferentiated
    • arthritis that does not meet criteria for other JIA subtypes
    • 11–21% of JIA cases

Reference:


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