Yamaguchi et al listed preliminary criteria for the diagnosis of adult Still's disease. The authors are from the Japanese Adult Still's Disease Research Committee.
Age at onset: >= 16 years
Major criteria:
(1) fever >= 39°C for >=1 week
(2) arthralgia >= 2 weeks
(3) typical rash (macular or maculopapular, nonpruritic, salmon-pink eruption that usually appears during fever)
(4) leukocytosis >= 10,000 per µL with >= 80% granulocytes
Minor criteria:
(1) sore throat
(2) lymphadenopathy and/or splenomegaly
(3) liver dysfunction
(4) negative rheumatoid factor (RF) and negative antinuclear antibodies (ANA)
Need to exclude:
(1) infection, including sepsis and infectious mononucleosis
(2) cancer, especially malignant lymphoma
(3) rheumatic disease, especially polyarteritis nodosa
A patient is diagnosed with adult Still's disease if:
(1) total number of criteria >= 5
(2) major criteria >= 2
(3) no other condition that can explain the findings better
Performance:
• The sensitivity was 96% and specificity 92% (Youden index 88%).
