Galve E, Ordi J, Candell J, Soler Soler J
Servicio de Cardiología, Hospital General Universitario Vall d'Hebron, Barcelona.
Rev Esp Cardiol. 1998 Mar;51(3):232-42. doi: 10.1016/s0300-8932(98)74738-4.
Connective tissue diseases encompass a wide group of nosologic entities of unknown etiology, characterized by multisystemic organ involvement, sharing an immunologic pathogenetic mechanism, producing a variety of inflammatory manifestations, and whose primary lesion is always a diffuse vasculitis. Any part of the cardiovascular system may be involved, including the pericardium, the myocardium, the endocardium and valves, the coronary arteries, the aorta, the pulmonary vasculature, the peripheral arteries, veins, arterioles, venules, and the capillary beds of almost every organ subsystem. Pathologic studies disclose a high prevalence of heart involvement, but the presence and extent of pathologic findings correlate poorly with clinical manifestations. With the advent of echocardiography-Doppler, milder and earlier cases are now recognized. Although these patients continue under the care of rheumatologists and internists, when cardiac involvement arises, cardiologists must be aware of the characteristics, outcome and management of connective tissue diseases.
结缔组织病包括一大类病因不明的疾病实体，其特征为多系统器官受累，具有共同的免疫发病机制，产生多种炎症表现，且其原发性病变始终是弥漫性血管炎。心血管系统的任何部位都可能受累，包括心包、心肌、心内膜和瓣膜、冠状动脉、主动脉、肺血管系统、外周动脉、静脉、小动脉、小静脉以及几乎每个器官子系统的毛细血管床。病理研究表明心脏受累的发生率很高，但病理结果的存在和程度与临床表现的相关性很差。随着超声心动图-多普勒技术的出现，现在能够识别出病情较轻和较早的病例。尽管这些患者仍由风湿病学家和内科医生诊治，但当出现心脏受累时，心脏病学家必须了解结缔组织病的特征、预后及治疗方法。
