Criteria
Yamaguchi classification criteria for AOSD[48]
At least five criteria are required to confirm a clinical diagnosis of AOSD, two or more of which must be major criteria AND any infection, malignancy, or other rheumatic disorder known to mimic AOSD must be excluded.
Major criteria
Fever of at least 39.0°C (102.2°F) lasting at least 1 week
Arthralgias or arthritis lasting 2 weeks or longer
A non-pruritic macular or maculopapular skin rash that is salmon-coloured in appearance and usually found over the trunk or extremities during febrile episodes
Leukocytosis (10,000/microlitre or greater), with at least 80% granulocytes
Minor criteria
Sore throat
Lymphadenopathy
Hepatomegaly or splenomegaly
Abnormal liver function studies, particularly elevations in aspartate and alanine aminotransferase and lactate dehydrogenase concentrations
Negative tests for antinuclear antibody (ANA) and rheumatoid factor (RF).
Fautrel classification criteria for AOSD[49]
The Fautrel criteria were developed to avoid the need for exclusion criteria but require testing of glycosylated ferritin, which is not available in many healthcare facilities. A confirmation of an AOSD diagnosis requires:
≥4 major criteria OR
three major criteria AND two minor criteria
Major criteria
Spiking fever ≥39.0°C (≥102.2°F)
Arthralgias
Transient erythematous rash
Pharyngitis
Polymorphonuclear neutrophils ≥80%
Glycosylated ferritin ≤20%
Minor criteria
Maculopapular rash
Leukocytes ≥10,000/microlitre (10 × 10⁹/L)
Use of this content is subject to our disclaimer