Atypical Presentation of Behçet Disease Unmasked by Acute Coronary Syndrome

Behçet disease is a rare multisystem vasculitis that may affect the coronary arteries, presenting as acute coronary syndrome (ACS) in young patients without risk factors. A 26-year-old man with recurrent oral ulcers experienced chest pain over 3 months. Coronary angiography revealed multivessel disease, and a subsequent angiogram showed coronary aneurysms, suggesting vasculitis. Behçet disease was diagnosed based on the International Criteria for Behçet's Disease and HLA-B51 positivity. The patient improved with corticosteroids and colchicine. In young patients presenting with ACS and no significant risk factors for atherosclerosis, Behçet disease should be considered as a potential cause of coronary artery vasculitis. Coronary aneurysms may be an associated finding. Vasculitis should be considered in the differential diagnosis for young patients with ACS without significant cardiovascular risk factors. Behçet disease can lead to coronary artery aneurysms; early recognition and management are critical.