Behçet’s syndrome

(Nat Rev Dis Primers 2021;7:67)

• Systemic vasculitis affecting all vessel sizes, venous and arterial, a/w painful oral and/or genital ulcers
• Epi: usually young adults (25–35 y); ♂ = ♀, ↑ severity in ♂; a/w HLA-B51; ↑ prev on old Silk Road (Turkey, Middle East, Asia) w/ 5 vs. 370/100,000 in U.S. vs. Turkey
• Classification criteria of Behçet’s syndrome
• Other clinical manifestations: most recur but are not chronic Arthritis: mild, ± symmetric, nondestructive, involving knees and ankles Neurologic: usually involvement of midbrain parenchyma; peripheral neuropathy rare Vascular: superficial or deep vein thrombosis (25%); arterial stenosis, occlusion, and aneurysm can also occur; low incidence of thromboembolism
• Dx studies: ↑ ESR/CRP; ulcer swab to r/o HSV; ulcer bx nonspecific; ophtho eval if sx

Treatment of Behçet’s syndrome (Ann Rheum Dis 2018;77:808)