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JIA Classification (ILAR)

ILAR Classification Criteria for Juvenile Idiopathic Arthritis

DiseaseCriteriaExclusions

Systemic Arthritis

Definite:

  1. Quotidian fever for at least 2 weeks
  2. Evanescent, non-fixed erythematous rash
  3. Arthritis

Probable:

In the absence of arthritis, 1 and 2 (above) plus any two of:

  1. General lymph node enlargement
  2. Hepatomegaly or splenomegaly
  3. Serositis
  • NOMIDa
  • Periodic syndromesb
  • Drug hypersensitivity

Polyarthritis RF-

Arthritis of ≥5 joints during the first 6 months of disease

  • Positive RFc

Polyarthritis RF+

  1. Arthritis of ≥5 joints during the first 6 months of disease
  2. Positive RF on at least two occasion 3 months apart
  • Psoriasis

Oligoarthritis

Persistent Oligoarthritis

Arthritis of 1-4 joints during the onset or course of the disease

  • Psoriasis
  • Positive RF

Extended Oligoarthritis

  1. Arthritis of 1-4 joints during the first 6 months of disease
  2. Arthritis of ≥5 joints after the first 6 months of disease
  • Psoriasis
  • Positive RF

Enthesitis-related arthritis

Arthritis and enthesitis or,

Arthritis or enthesitis with at least two of:

  1. Sacroiliac joint tenderness and/or inflammatory spinal pain
  2. Presence of HLA B27
  3. Family history in at least one first- or second-degree relative of medically confirmed HLA B-27-associated disease
  4. Anterior uveitis that is usually associated with pain, redness or photophobia
  5. Onset of arthritis in a boy after 8 years of age
  • Psoriasis confirmed by a dermatologist in at least one first- or second-degree relative
  • Presence of systemic arthritis

Psoriatic arthritis

Arthritis and psoriasis or,

Arthritis and at least two of:

  1. Dactylitis
  2. Nail pitting or onycholysis
  3. Family history of psoriasis in a first-degree relative
  • Positive RF

Other

Arthritis for ≥6 months and does not fit into any of the above categories or fits into more than 1 category

  • a Neonatal onset multi-system inflammatory disease
  • b Including familial Mediterranean fever, hyper-IgD syndrome, FAPA (fever, aphthous ulceration, pharyngitis, adenopathy)
  • c RF = Rheumatoid factor

Source: ILAR classification of juvenile idiopathic arthritis; see https://www.researchgate.net/figure/LAR-Classification-criteria-for-the-idiopathic-arthritis-of-childhood-16_tbl1_237562872