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. 2022 Jul 18;11(14):2231. doi: 10.3390/cells11142231

Table 7.

Classification criteria of systemic juvenile idiopathic arthritis (according to PRINTO organization): fever has to be associated with 2 major criteria or with 1 major criterion and 2 minor criteria, after exclusion of infectious, neoplastic, autoimmune and hereditary autoinflammatory diseases.

Cardinal Sign Major Criteria Minor Criteria
Fever of unknown origin that is documented to be daily (until 39 °C once a day with intermittent course) for at least 3 consecutive days and reoccurring over an observation period of at least two weeks
  • (a)

    evanescent nonfixed erythematous rash

  • (b)

    arthritis
  • (1)

    generalized lymph node enlargement and/or hepatomegaly and/or splenomegaly

  • (2)

    serositis

  • (3)

    arthralgia lasting 2 weeks or longer (in the absence of arthritis)

  • (4)

    leukocytosis (≥15,000/mm3) with neutrophilia