Adult Onset Still's Disease


Yamaguchi Criteria for Adult Onset Still's Disease

Major criteria

Temperature of ≥39℃ for ≥1 week
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Leukocytosis ≥10,000/mm3 with ≥80% granulocytes
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Typical rash

Macular or maculopapular nonpruritic salmon-pink eruption usually appearing during fever

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Arthralgia lasting ≥2 weeks
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Minor criteria

Sore throat
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Lymphadenopathy and/or Splenomegaly

Lymphadenopathy is defined as recent development of significant lymph node swelling, and splenomegaly is confirmed on palpation or by imaging

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Liver dysfunction

Liver dysfunction is defined as an abnormally elevated levels of transaminases and/or lactate dehydrogenase, which is attributed to liver damage associated with this disease but not with drug allergy/toxicity or other causes. For the differentiation, it is recommended to see if liver dysfunction returns to normal upon discontinuation of hepato-toxic drug or not, before applying this criterion.

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Negative RF and negative ANA

RF is serum must be negative by routine test for the detection of IgM RF, and serum ANA must be negative by routine immunofluoresence test. ANA = antinuclear antibody RF = rheumatoid factor

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Exclusion criteria

Are the following conditions excluded?

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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Sensitivity: 98.5%

Specificity: 91.6%


The tool is for educational use only, is not meant to be a substitute for professional advice and should not be used for medical diagnosis and/or medical treatment.


International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). The International Criteria for Behçet's Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014 Mar;28(3):338-47. doi: 10.1111/jdv.12107. Epub 2013 Feb 26. PMID: 23441863.

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