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Behcet syndrome is a rare vasculitis that affects both arteries and veins. Vasculo-Bechet Syndrome (VBS) is seen predominantly in men. Genetic predisposition and immune dysregulation leading to inflammation, endothelial damage, and impaired fibrinolysis contribute to its pathogenesis. Isolated case reports of Behcet syndrome (BS) with associated acute coronary syndrome (ACS) have been reported in the past. In this study, we present the first systematic review of such cases. A systematic search was conducted using Pubmed, Google Scholar, CINAHL, Cochrane CENTRAL, and Web of Science databases from 1980-2018 to identify case reports of myocardial infarction associated with BS. Cases that fulfilled the criteria for BS were selected for analysis. Demographic data, electrocardiography, echocardiography, angiography findings, and management were analyzed when available. We identified 62 case reports. Most subjects were men with a mean age of 37 years. Twenty-one percent were smokers, but other traditional cardiovascular risk factors were less common. Myocardial infarction was confirmed in half of the cases with findings on electrocardiogram (ECG). Echocardiogram revealed wall motion abnormality in 76% of patients, and angiography showed double-vessel disease in more than half of the cases. Mortality was reported in 1.6% of the cases. This systematic review shows that ACS in BS affects young males with low prevalence of coronary artery disease risk factors. Chest pain is the most common presenting feature and ST-segment elevation myocardial infarction (STEMI) was the most common ECG finding. Immunotherapy may be helpful to prevent future ACS in these patients.
Cite this article as: Kariyanna PT, Shah P, Jayarangaiah A, Chowdhury YS, Lazaro D. Acute coronary syndrome in Behcet’s syndrome: A systematic review. Eur J Rheumatol 2021; 8(1): 31-5.