Hints for Genetic and Clinical Differentiation of Adult-Onset Monogenic Autoinflammatory Diseases
Table 3
Clinical criteria for the diagnosis of adult onset Still’s disease (AOSD); adapted from Yamaguchi et al. [132] and Fautrel et al. [129]. Infections, malignancies, and other rheumatologic diseases represent exclusion criteria.
Yamaguchi criteria
Fautrel criteria
Major criteria
Major criteria
(i) , intermittent, lasting at least one week
(i)
(ii) Arthralgia for at least 2 weeks
(ii) Arthralgia
(iii) Typical skin rash
(iii) Transient erythema
(iv) with
(iv) Pharyngitis
(v)
(vi)
Minor criteria
Minor criteria
(i) Sore throat
(i) Maculopapular skin rash
(ii) Lymphadenopathy and/or splenomegaly
(ii)
(iii) Abnormal liver function tests
(iv) Negative antinuclear antibodies and rheumatoid factor
At least 5 items, including 2 major criteria, are requested
4 major criteria or 3 major+2 minor items are requested