Paris Descartes University, Department of Rheumatology, Cochin Hospital, Paris, France.
Clin Exp Rheumatol. 2010 Sep-Oct;28(5 Suppl 62):S48-53. Epub 2010 Nov 3.
Systemic sclerosis (SSc) is a connective tissue disease characterised by widespread vascular lesions and fibrosis of the skin and internal organs. Cardiac involvement is recognised as a poor prognostic factor when clinically evident. Primary myocardial involvement is common in SSc. Increasing evidence strongly suggests that myocardial involvement is related to repeated focal ischaemia leading to myocardial fibrosis with irreversible lesions. Reproducible data have shown that this relates to microcirculation impairment with abnormal vasoreactivity, with or without associated structural vascular abnormalities. Consistently, atherosclerosis and macrovascular coronary lesions do not seem to be increased in SSc. Myocardial involvement leads to abnormal systolic and diastolic left ventricular dysfunction and right ventricular dysfunction. Sensitive and quantitative methods have demonstrated the ability of vasodilators, including calcium channel blockers and angiotensin converting enzyme inhibitors, to improve both perfusion and function abnormalities further emphasising the critical role of microcirculation impairment. Recent quantitative methods such as tissue Doppler echocardiography and magnetic resonance imaging have underlined these results.
系统性硬化症（SSc）是一种结缔组织疾病，其特征为广泛的血管病变和皮肤及内脏器官的纤维化。临床上明显的心脏受累被认为是预后不良的因素。原发性心肌受累在 SSc 中很常见。越来越多的证据强烈表明，心肌受累与反复局部缺血有关，导致心肌纤维化和不可逆转的病变。可重复的数据表明，这与微循环损伤有关，伴有或不伴有相关的结构性血管异常，血管反应异常。一致的是，动脉粥样硬化和大血管冠状动脉病变似乎在 SSc 中没有增加。心肌受累导致左心室收缩和舒张功能异常以及右心室功能障碍。敏感和定量的方法已经证明，包括钙通道阻滞剂和血管紧张素转换酶抑制剂在内的血管扩张剂能够改善灌注和功能异常，进一步强调了微循环损伤的关键作用。最近的定量方法，如组织多普勒超声心动图和磁共振成像，强调了这些结果。
