Review Article
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 |
|
|