Description

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%).


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