Hints for Genetic and Clinical Differentiation of Adult-Onset Monogenic Autoinflammatory Diseases

Table 6

Criteria for the diagnosis of Behçet’s disease (BD): International Study Group Criteria (ISGC) [152] and International Criteria for BD (ICBD) [153].

ISGC	ICBD
Mandatory item	Items	Score
(i) Recurrent oral aphthosis (at least 3 episodes per year)	(i) Oral aphthosis	2
Additional items	(ii) Genital aphthosis	2
(i) Recurrent genital ulcers; (ii) Ocular or retinal inflammatory manifestations; (iii) Skin lesions (papulopustular lesions, erythema nodosum); (iv) Positive pathergy reaction	(iii) Ocular inflammatory lesions	2
	(iv) Skin lesions	1
	(v) Neurological involvement	1
	(vi) Vascular manifestations	1
	(vii) Positive pathergy test	1
At least 2 additional items are requested	Cut-off	≥4