Villiger Peter M
Klinik für Rheumatologie und Klinische Immunologie & Allergologie, Universitätsspital, Bern.
Ther Umsch. 2008 May;65(5):283-7. doi: 10.1024/0040-5930.65.5.283.
Systemic sclerosis (scleroderma) is characterized by autoimmune phenomena and a progressive fibrosis. Clinical signs include Raynaud's phenomenon, pathognomonic nail fold capillary changes, pulmonal, renal, cardial and intestinal involvement. The two forms of systemic sclerosis, the diffuse and the limited show distinct disease phenomena and evolution. The diffuse form typically leads to a rapid sclerosis of the whole skin, it is associated with the auto-antibody Scl-70, it presents with an interstitial fibrosis of the lung, myositis and cardial fibrosis, a progressive loss of intestinal motility with maldigestion and it bears the risk of renal crisis. The limited form is characterized by sclerosis of the skin of the distal parts of the limbs and the face only, it is associated with the centromer auto-antibody and a much slower progression of visceral fibrosis. A typical late stage manifestation is pulmonary arterial hypertension. New drugs have improved quality of life. Their correct indication is based on a systematic monitoring of disease.
系统性硬化症（硬皮病）的特征是自身免疫现象和进行性纤维化。临床症状包括雷诺现象、具有诊断意义的甲襞毛细血管变化、肺部、肾脏、心脏和肠道受累。系统性硬化症的两种类型，即弥漫型和局限型，表现出不同的疾病现象和病程。弥漫型通常导致全身皮肤迅速硬化，与自身抗体Scl - 70相关，表现为肺间质纤维化、肌炎和心脏纤维化、肠道蠕动逐渐丧失伴消化功能不良，并有发生肾危象的风险。局限型的特征是仅四肢远端和面部皮肤硬化，与着丝点自身抗体相关，内脏纤维化进展要慢得多。典型的晚期表现是肺动脉高压。新药改善了生活质量。其正确应用基于对疾病的系统监测。
