心脏白塞病二例及文献复习  

Spectrum of cardiac lesions in Beh^et disease： two cases report and review of the literature

作　　者：袁风红[1] 邹耀红[1] 高恺言[1] 俞可佳[1] 胥魏[1] 胡赟霞[1] 刘婷[1] 时一添[1] 

机构地区：[1]南京医科大学附属无锡市人民医院风湿免疫科,214023

出　　处：《中华风湿病学杂志》2015年第3期185-191,共7页Chinese Journal of Rheumatology

摘　　要：目的探讨心脏白塞病的临床特点及诊治。方法分析2例心脏白塞病的临床特点；并搜索Medline和维普数据库复习相关文献。结果2例患者均有主动脉瓣瓣膜脱垂伴关闭不全，其中1例尚有二尖瓣脱垂伴关闭不全，2例患者均未发现狭窄表现。瓣膜病理主要表现为退变、变性、纤维化。国内外文献表明，白塞病心脏累及以男性多见，可表现为瓣膜累及、心包积液、心内占位、冠状动脉累及、心肌炎、心内膜下心肌纤维化及室壁瘤等。其中主动脉瓣累及关闭不全是心脏白塞病的常见表现。心脏白塞病的内科治疗包括糖皮质激素、免疫抑制剂、秋水仙碱及抗凝治疗。心脏病情进行性恶化时需考虑手术治疗。结论心脏白塞病是白塞病的少见重症表现，预后欠佳，早期积极内科免疫抑制治疗及改良的外科手术治疗有助于改善预后。Objective To further understand the clinical characteristics of cardiac involvement in Behqet disease （BD）. Methods Two cases of BD with cardiac damage were reported and analyzed. And literatures from Medline database and Weipu database were reviewed and discussed. Results Two patients had aortic valve prolapse with valve insufl3eiency. One patient had mitral valve prolapse with mitral valve insufficiency. Valve stenosis was not found in these two cases. Pathologically, aortic valves showed fibrosis and denaturation. Review of the literature showed that patients with cardiac involvement were more frequently male. Cardiac abnormalities include endoearditis with valvular regurgitation, pericarditis, intracardiac thrombosis, coronary arteritis with or without myocardial infarction, myocarditis, endomyocardial fibrosis and ventricular aneurysm. The most frequent clinical features were aortic valve insufficiency. Medical treatment of cardiac involvement included corticosteroids,colchicine, immunosuppressants, oral anticoagulation and/or low-dose aspirin. Surgery was performed in some cases. Conclusion The cardiac involvement in BD is a rare but poor prognosis sign that requires timely diagnosis and management. The immunosuppressive therapy and Bentall type operation may be essential for improving the treatment outcome.

关 键 词：贝赫切特综合征 回顾性研究 临床特点 治疗 

分 类 号：R597.9[医药卫生—内科学]