Cardiac Manifestations in Behçet’s Syndrome
Federica Bello, Giacomo Bagni, Emire Seyahi, Emanuele Chiara, Iacopo Olivotto, David Saadoun, Giacomo Emmi
Abstract
PURPOSE OF THE REVIEW: Behçet's Syndrome (BS) is a multisystemic vasculitis that can affect the heart, leading to pericarditis, myocarditis, intracardiac thrombosis, endomyocardial fibrosis, valvular dysfunction, and coronary artery disease. This review summarizes the clinical presentation, diagnostic challenges, and therapeutic strategies for cardiac involvement in BS. RECENT FINDINGS: Advanced imaging techniques have revealed subclinical cardiac involvement in BS. Myocardial dysfunction and fibrosis contribute to heart failure and arrhythmias, while intracardiac thrombi often coexist with pulmonary artery involvement. Coronary artery vasculitis and aneurysms may mimic atherosclerotic disease, complicating diagnosis. Biologic therapies, including TNF-α inhibitors, show promise in refractory cases. Early diagnosis and immunosuppressive therapy are crucial. A multidisciplinary approach is essential to managing cardiac complications and optimizing patient outcomes. Future research should refine screening protocols and explore targeted immunotherapies for BS-related cardiovascular disease.