Cocco Giuseppe, Gasparyan Armen Yuri
Cardiology Office, CH-4310 Rheinfelden, Switzerland.
Open Cardiovasc Med J. 2010 Feb 23;4:63-70. doi: 10.2174/1874192401004020063.
Behçet's disease (BD) is an enigmatic inflammatory disorder, with vasculitis (perivasculitis) underlying pathophysiology of its multisystemic affections. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognized that cardiac involvement and arterial complications (aneurysms, pseudoaneurysms, rupture and thrombosis) are important part of the course of BD. Pericarditis, myocardial (diastolic and/or systolic dysfunction), valvular and coronary (thrombosis, aneurysms, rupture) involvement, intracardiac thrombi (predominantly right-sided) are, probably, the most frequent cardiac manifestations. Treatment of cardiovascular involvement in BD is largely empirical and aimed at suppression of vasculitis. The most challenging seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleedings. Cardiologists should always bear in mind potential threats of (a)symptomatic cardiovascular involvement in BD.
白塞病(BD)是一种神秘的炎症性疾病,其多系统损害的病理生理学基础为血管炎(血管周围炎)。静脉病变和血栓形成并发症是白塞病的特征。然而,人们越来越认识到心脏受累和动脉并发症(动脉瘤、假性动脉瘤、破裂和血栓形成)是白塞病病程的重要组成部分。心包炎、心肌(舒张和/或收缩功能障碍)、瓣膜和冠状动脉(血栓形成、动脉瘤、破裂)受累、心内血栓(主要在右侧)可能是最常见的心脏表现。白塞病心血管受累的治疗很大程度上是经验性的,旨在抑制血管炎。由于存在出血相关风险,动脉动脉瘤和血栓的治疗似乎最具挑战性。心脏病专家应始终牢记白塞病有(无)症状心血管受累的潜在威胁。