Kahan A, Coghlan G, McLaughlin V
Department of Rheumatology A, Cochin Hospital, University of Paris Descartes, AP-HP, Paris, France.
Rheumatology (Oxford). 2009 Jun;48 Suppl 3:iii45-8. doi: 10.1093/rheumatology/kep110.
The majority of patients with SSc are believed to have subclinical primary cardiac involvement. Overt cardiac manifestations of SSc are associated with poor prognosis and can be difficult to manage. Primary myocardial disease, i.e. without systemic or pulmonary hypertension and without significant pulmonary or renal disease, is postulated to be due to microvascular ischaemia. Undetected early cardiac manifestations can progress silently to myocardial fibrosis. Symptoms may manifest without warning and can rapidly lead to arrhythmia and left and right heart dysfunction and failure. Of the currently practical screening methods, annual echocardiography and/or evaluation of N-terminal portion of pro-B-type natriuretic peptide concentrations should therefore be employed in SSc patients, in order to anticipate the development of cardiac symptoms. Although there is limited evidence in respect of specific therapeutic options, treatment of early abnormalities with calcium channel blockers and angiotensin-converting enzyme inhibitors may improve myocardial perfusion and function, while standard management of overt cardiac disease is equally appropriate in the SSc population. However, it remains to be seen if early intervention can limit the progression of these life-threatening complications.
大多数系统性硬化症（SSc）患者被认为存在亚临床原发性心脏受累情况。SSc的明显心脏表现与预后不良相关，且可能难以处理。原发性心肌病，即无系统性或肺动脉高压且无明显肺部或肾脏疾病，据推测是由微血管缺血所致。未被检测到的早期心脏表现可无声地进展为心肌纤维化。症状可能毫无征兆地出现，并可迅速导致心律失常以及左右心功能障碍和衰竭。因此，就目前实用的筛查方法而言，SSc患者应每年进行超声心动图检查和/或评估B型利钠肽原N端浓度，以便预测心脏症状的发展。尽管关于具体治疗方案的证据有限，但使用钙通道阻滞剂和血管紧张素转换酶抑制剂治疗早期异常情况可能会改善心肌灌注和功能，而对于明显心脏病的标准管理在SSc人群中同样适用。然而，早期干预能否限制这些危及生命的并发症的进展仍有待观察。
