进行性系统性硬化症的皮肤外表现

The Extra-skin Manifestation of Progressive Systemic Sclerosis

本文报告56例PSS中,95％的患者有脏器受累,肺部病变最多见,心和消化道次之。首发症状以雷诺氏征为多。发热、关节痛(关节炎)、肌炎常于发病1～5年内发生。REST综合征患者病程较长。合并SS者可有多脏器受累。无严重的心、肾和血液系统受累者,预后良好。

This article reports 56 cases of PSS. Ninetyfive percent of the patients had one or multiple organ involvements in which the lungs were the most commonand heart and digestive tract followed. Raynaund's phenomenon was the most common initial symptoms fever, axthralgia (arthritis) and mysitis were occured mostly in one to five years after the onset of the disease. The patients with REST syndrome had a long course. The PSS patients with sjgren's syndrome had multiple organ lesion. The better prognosis revealed in those patients had no involvement of the heart. Kedney and blood system.