Pagnoux C, Guillevin L
Department of Internal Medicine, Hôpital Cochin, Université Paris, Paris, France.
Lupus. 2005;14(9):718-22. doi: 10.1191/0961203305lu2207oa.
The heart can be involved in vasculitides but the frequency of its involvement and the manifestations vary according to the vasculitis. Cardiovascular manifestations include cardiomyopathy (specific or resulting from myocardial infarctions), coronary arteritis (with risk of aneurysms, stenoses and thrombosis formation or rupture), pericarditis, valvulitis, conduction-tissue involvement (with heart blocks), arrhythmias (mainly supraventricular) and/or dissection of the aorta (and/or its proximal branches). As many of these manifestations are clinically silent, at least during their early stages, heart function should be systematically assessed in vasculitis patients, with at least ECG and echocardiography, and more invasive exploratory procedures when the former reveal abnormalities or symptoms become manifest. Specific cardiomyopathy has been identified as a factor of poor outcome in small and medium-sized vessel vasculitides (five-factor score). Therefore, in addition to symptomatic treatments, prescription of corticosteroids and immunosuppressants (mainly cyclophosphamide) is considered mandatory. This regimen has dramatically improved the overall prognosis of affected patients.
心脏可受累于血管炎，但受累频率及表现因血管炎类型而异。心血管表现包括心肌病（特异性心肌病或心肌梗死所致心肌病）、冠状动脉炎（有动脉瘤、狭窄、血栓形成或破裂风险）、心包炎、瓣膜炎、传导组织受累（伴心脏传导阻滞）、心律失常（主要为室上性）和/或主动脉（及其近端分支）夹层。由于这些表现中许多在临床上并无症状，至少在早期阶段如此，因此对于血管炎患者应系统评估心脏功能，至少进行心电图和超声心动图检查，当前者显示异常或出现症状时，则需进行更具侵入性的检查。特异性心肌病已被确定为中小血管血管炎（五因素评分）预后不良的一个因素。因此，除对症治疗外，使用皮质类固醇和免疫抑制剂（主要是环磷酰胺）被认为是必要的。这种治疗方案显著改善了受累患者的总体预后。
