| Major criteria |
1) Fever ≥39 degrees Celsius persisting for ≥1 week 2) Arthralgia/arthritis persisting for ≥2 weeks 3) Typical rash 4) White blood cell count ≥10×109/L (>80% neutrophils) |
|
Minor criteria |
1) Sore throat 2) Lymphadenopathy and/or splenomegaly 3) Increased serum aminotransferase or lactate dehydrogenase levels (after other causes have been excluded) 4) Negative IgM rheumatoid factor and antinuclear antibodies (immunofluorescence assay) |
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Exclusion criteria |
1) Infections, in particular sepsis and infectious mononucleosis 2) Malignancy, in particular lymphoma 3) Other rheumatic diseases, in particular polyarteritis nodosa and vasculitis in the course of rheumatoid arthritis |
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For the diagnosis of adult-onset Still disease, ≥5 criteria must be met, including ≥2 major. In patients with any of the exclusion criteria, the diagnosis is excluded. Sensitivity of the criteria is 80.6%, and specificity is 98.5%. |
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Source: J Rheumatol. 1992;19(3):424-30. |
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| Notes: An alternative diagnostic score by Fautrel, without exclusion criteria, is available at www.journals.lww.com. According to this scale, ≥4 major criteria must be met for adult-onset Still disease to be considered (spiking fever of ≥39 degrees Celsius, arthralgia, transient erythema, pharyngitis, polymorphonuclear neutrophil count of ≥80%, glycosylated ferritin of <20%); or 3 major and 2 minor criteria (maculopapular rash and white blood cell count of ≥10×109/L). | |