Launay David, Hachulla Eric
Service de médecine interne CHRU-Hôpital Claude-Huriez 59037 Lille.
Rev Prat. 2002 Nov 1;52(17):1901-7.
Systemic sclerosis is a connective tissue disease characterised by microvascular involvement and fibrosis affecting skin, lung, heart and intestinal tract. Sixty percent of deaths are secondary to heart or lung involvement and (or) to pulmonary hypertension. Clinical expression of these complications is present only in patients with the most severe problems. Therefore, it is appropriate to systematically and repeatedly look for these complications, even in asymptomatic patients. Heart involvement can present as cardiomyopathy, pericarditis, conduction or repolarisation abnormalities and arrhythmia, sometimes leading to sudden death. Pulmonary hypertension is due either to direct involvement of pulmonary arteries or is secondary to lung fibrosis. Doppler echocardiography and lung function tests are systematically and repeatedly proposed to screen patients for pulmonary hypertension at initial diagnosis and during follow-up. Right heart catheterisation ascertains the diagnosis of pulmonary hypertension. Chronic pulmonary interstitial fibrosis is the most frequent manifestation of lung involvement. High resolution computed tomography is the method of choice to make the diagnosis and to assess the severity of the lesions. Treatment is not completely standardised. However, a severe and rapidly progressing lung involvement is probably a good indication of immunosuppressive agents, such as cyclophosphamide.
系统性硬化症是一种结缔组织疾病，其特征为微血管受累以及影响皮肤、肺、心脏和肠道的纤维化。60%的死亡继发于心脏或肺部受累及（或）肺动脉高压。这些并发症的临床表现仅出现在问题最为严重的患者中。因此，即使对于无症状患者，也应系统且反复地排查这些并发症。心脏受累可表现为心肌病、心包炎、传导或复极异常以及心律失常，有时会导致猝死。肺动脉高压要么是由于肺动脉直接受累，要么是继发于肺纤维化。在初始诊断及随访期间，会系统且反复地建议采用多普勒超声心动图和肺功能测试来筛查肺动脉高压患者。右心导管检查可确诊肺动脉高压。慢性肺间质纤维化是肺部受累最常见的表现。高分辨率计算机断层扫描是进行诊断及评估病变严重程度的首选方法。治疗尚未完全标准化。然而，严重且进展迅速的肺部受累可能是使用免疫抑制剂（如环磷酰胺）的良好指征。
