Bello Federica, Bagni Giacomo, Seyahi Emire, Chiara Emanuele, Olivotto Iacopo, Saadoun David, Emmi Giacomo
Department of Experimental and Clinical Medicine, University of Florence, Italy and Internal Interdisciplinary Unit, Behçet Centre, Careggi University Hospital, Florence, Italy.
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy.
Curr Rheumatol Rep. 2025 Jul 17;27(1):31. doi: 10.1007/s11926-025-01190-z.
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.
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.
白塞病（BS）是一种多系统血管炎，可累及心脏，导致心包炎、心肌炎、心内血栓形成、心内膜纤维化、瓣膜功能障碍和冠状动脉疾病。本综述总结了白塞病心脏受累的临床表现、诊断挑战和治疗策略。
先进的成像技术已揭示白塞病存在亚临床心脏受累。心肌功能障碍和纤维化会导致心力衰竭和心律失常，而心内血栓常与肺动脉受累并存。冠状动脉血管炎和动脉瘤可能类似动脉粥样硬化疾病，使诊断复杂化。包括肿瘤坏死因子-α抑制剂在内的生物疗法在难治性病例中显示出前景。早期诊断和免疫抑制治疗至关重要。多学科方法对于管理心脏并发症和优化患者预后必不可少。未来的研究应完善筛查方案，并探索针对白塞病相关心血管疾病的靶向免疫疗法。
