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