Table 1 Selected criteria for systemic juvenile idiopathic arthritis and adult-onset Still’s disease
Disease | AOSD | Systemic JIA | Systemic JIA | Systemic JIA |
|---|---|---|---|---|
Lead author or sponsor | Yamaguchi | ILAR | CARRA | PRINTO |
Publication date | 1992 | 2004 | 2012 | 2019 |
Inclusion criteria | 5 criteria, including at least 2 major criteria Major criteria • fever ≥ 39C ≥ 1 week • arthralgia ≥ 2 weeks • typical rash • WBC ≥ 10 k including ≥ 80% granulocytes Minor criteria • sore throat • lymphadenopathy and/or splenomegaly • liver dysfunction • negative RF and ANA | Arthritis of unknown etiology beginning before the 16th birthday and persisting ≥ 6 weeks Fever ≥ 2 weeks, daily ≥ 3d Plus one or more of • evanescent erythematous rash • generalized lymphadenopathy • hepatomegaly and/or splenomegaly • serositis | Arthritis beginning between age 6 months and 18y of age (before 19th birthday) and persisting ≥ 1 week Fever ≥ 2 weeks, must exhibit quotidian pattern at some point Plus one or more of • evanescent erythematous rash • generalized lymphadenopathy • hepatomegaly or splenomegaly • pericarditis, pleuritis and/or peritonitis | Onset before 18th birthday Fever ≥ 2 weeks, daily ≥ 3d + 2 major criteria or 1 major / 2 minor criteria Major criteria • evanescent erythematous rash • arthritis (any duration) Minor criteria • generalized lymphadenopathy, hepato- and/or splenomegaly • serositis • arthralgia ≥ 2wk • WBC ≥ 15 k with neutrophilia |
Exclusion criteria | Infections (especially sepsis and mononucleosis), malignancy (especially lymphoma), rheumatic disease (especially vasculitis) | HLA-B27 + and onset ≥ age 6y, RF twice ≥ 3 months apart, personal or 1st degree family history of psoriasis, ankylosing spondylitis, enthesitis related arthritis, sacroiliitis with inflammatory bowel disease, Reiter’s, acute anterior uveitis | infection, malignancy, or other causes that can present with similar symptoms to systemic JIA | other known conditions |
Notes | Age of onset not specified | Arthritis must be observed by a physician; duration ≥ 1 week specified in ref. (24) | PRINTO definition of JIA requires inflammatory disease to persist ≥ 6 weeks |