Criteria

Diagnostic criteria for juvenile idiopathic arthritis is a complex and changing area that includes debate around the utility of classifying different patient groups by historical, biological, or phenotypic criteria and the continuum (or otherwise) with adult rheumatological disease.[56]

International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis[1]

JIA can be diagnosed if age at onset is under 16 years, disease duration is 6 weeks or greater, and other known conditions are excluded. The seven different categories are as follows.

  • Systemic arthritis is diagnosed if there is arthritis in 1 or more joints with, or preceded by, fever of at least 2 weeks' duration. Signs or symptoms must have been documented daily for at least 3 days and accompanied by 1 or more of the following: evanescent rash, generalised lymphadenopathy, hepato/splenomegaly, serositis. (Exclusions are A, B, C, and D from the exclusion list below.)

  • Oligoarthritis is diagnosed if there is arthritis affecting 1 to 4 joints during the first 6 months. Persistent oligoarthritis affects up to 4 joints throughout the course of the disease, and extended oligoarthritis affects more than 4 joints after the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)

  • Polyarthritis (rheumatoid factor [RF]-negative) is diagnosed if there is RF-negative arthritis affecting 5 or more joints during the first 6 months of disease. (Exclusions are A, B, C, D, and E from the exclusion list below.)

  • Polyarthritis (RF-positive) is diagnosed if there is RF-positive arthritis affecting 5 or more joints during the first 6 months of disease. Two or more RF tests (taken at least 3 months apart) are positive during the first 6 months of disease. (Exclusions are A, B, C, and E from the exclusion list below.)

  • Psoriatic arthritis is diagnosed if there is arthritis and psoriasis, or arthritis and at least 2 of the following: dactylitis, nail pitting, onycholysis, and/or family history of psoriasis (in a first-degree relative). (Exclusions are B, C, D, and E from the exclusion list below.)

  • Enthesitis-related arthritis is diagnosed if there is arthritis and/or enthesitis with at least 2 of the following: presence or history of sacroiliac joint tenderness with or without inflammatory lumbosacral pain; presence of HLA B27 antigen; onset of arthritis in a male over 6 years of age; acute (symptomatic) anterior uveitis; history of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter's syndrome, or acute anterior uveitis in a first-degree relative. (Exclusions are A, D, and E from the exclusion list below.)

  • Undifferentiated arthritis is diagnosed if there is arthritis that does not fulfil criteria in any of the above categories or that fulfils criteria for two or more of the above categories.

Exclusions:

  • A. Psoriasis or history of psoriasis in patients or first-degree relatives.

  • B. Arthritis in HLA B27 positive males beginning after the age of 6 years.

  • C. Ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, Reiter's syndrome, acute anterior uveitis, or history of one of these disorders in first-degree relatives.

  • D. Presence of IgM rheumatoid factor on at least two occasions at least 3 months apart.

  • E. Presence of systemic-onset juvenile idiopathic arthritis (SoJIA) in patients.

American College of Rheumatology criteria for juvenile rheumatoid arthritis (JRA)[2]

JRA can be diagnosed if age at onset is under 16 years; there is arthritis in 1 or more joints; disease duration is 6 weeks or greater; and other forms of juvenile arthritis (e.g., psoriatic and inflammatory bowel disease-associated arthritis) have been excluded.

Disease type is defined by the type of disease present in the first 6 months.

  • Systemic-onset JRA is daily (quotidian) fever spiking to more than 39°C (102.2°F) for 2 weeks or greater in association with arthritis of 1 or more joints.

  • Pauciarticular JRA is arthritis in 4 or fewer joints in the first 6 months of disease.

  • Polyarticular JRA is arthritis in 5 or more joints in the first 6 months of disease.

The European League Against Rheumatism criteria for juvenile chronic arthritis (JCA)[3]

JCA can be diagnosed if age at onset is under 16 years, there is arthritis in 1 or more joints, and disease duration is 3 months or greater.

Diagnostic criteria for specific disease types are outlined below.

  • Systemic JCA is arthritis with characteristic fever.

  • Pauciarticular JCA is arthritis in fewer than 5 joints.

  • Polyarticular JCA is arthritis in more than 4 joints with negative rheumatoid factor.

  • Juvenile rheumatoid arthritis is arthritis in more than 4 joints with positive rheumatoid factor.

  • Juvenile ankylosing spondylitis is the presence of features of ankylosing spondylitis in a child under 16 years old.

  • Juvenile psoriatic arthritis is the presence of psoriatic arthritis in a child under 16 years old.

The Pediatric Rheumatology International Trials Organization proposed classification[4]

This classification is proposed as a successor to the ILAR classification.

  • A. Systemic JIA - most closely related to adult-onset Still's disease

  • B. RF-positive JIA - most closely related to rheumatoid arthritis in adults

  • C. Enthesitis/spondylitis-related JIA - most closely related to the spondyloarthropathies

  • D. Early-onset antinuclear antibody-positive JIA - a disease course with distinctive clinical features in children

  • E. Other JIA

  • F. Unclassified JIA

Use of this content is subject to our disclaimer