Primary angiitis of the CNS (PACNS) and Behçet disease

Table 3 Diagnostic Criteria (International Team for the Revision of the International Criteria for Behcet’s 2014)

Criteria	Points
Eye involvement (Uveitis/Iritis with hypopyon, Retinitis)	2
Genital ulcerations (usually heals with scars)	2
Oral ulcerations (usually 3 x per year, no sequelae)	2
Skin lesions (Erythema nodosum, folliculitis, sterile pustulae)	1
Neuro-Behçet (no isolated headache)	1
Vascular manifestation (venous or arterial thromboses, aneurysms)	1
Positive pathergy test (optional)	1

4 Points: possible, 5 Points: very probable, 6 Points: almost certain diagnosis (94% sensitivity, 90% specificity)

ISSN: 2524-3489