Table 3

Yamaguchi criteria for diagnosis of adult-onset Still’s disease49

Major criteriaMinor criteria

  • Fever ≥39°C for ≥1 week

  • Arthralgia for ≥2 weeks

  • Typical rash

  • Leucocytosis (>10×10⁹/L) with >80% granulocytes

  • Lymphadenopathy and/or splenomegaly

  • Hepatic dysfunction (elevated serum aminotransferases)

  • Sore throat

  • Negative rheumatoid factor and antinuclear antibodies

Classification requires at least five criteria, of which two must be major.
Exclusion criteria include infection, malignancy and rheumatic diseases.