Zuber J P, Chizzolini C, Leimgruber A, Bart P A, Spertini F
Service d'immunologie et d'allergie Département de médecine CHUV, BH 18/707.
Rev Med Suisse. 2006 Apr 19;2(62):1058, 1060-6.
Systemic sclerosis (scleroderma) is considered as the most severe connective tissue disease. It is characterized by an abnormal immune activation, a vasculopathy and a fibrosis of the skin and of multiple internal organs. Numerous progress in the understanding of the pathogenesis with identification of key molecules have permit to introduce novel treatments that improve the management of some aspects of the disease. ACE inhibitors are effective in resolving renal crisis. Cyclophosphamide is useful for treatment of fibrosing alveolitis. Prostaglandins, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors permit to improve the treatment of the vascular complications (digital ulcerations, pulmonary arterial hypertension) of scleroderma.
系统性硬化症（硬皮病）被认为是最严重的结缔组织疾病。其特征为异常的免疫激活、血管病变以及皮肤和多个内脏器官的纤维化。随着关键分子的鉴定，在发病机制的理解方面取得了诸多进展，从而得以引入新的治疗方法，改善了该疾病某些方面的管理。血管紧张素转换酶抑制剂对解决肾危象有效。环磷酰胺可用于治疗纤维化肺泡炎。前列腺素、内皮素受体拮抗剂和5型磷酸二酯酶抑制剂有助于改善硬皮病血管并发症（指端溃疡、肺动脉高压）的治疗。
