Luderschmidt C, Kaulertz I, König G, Leisner B
Dtsch Med Wochenschr. 1984 Sep 14;109(37):1389-97. doi: 10.1055/s-2008-1069383.
The course of progressive systemic scleroderma was analysed from data collected on 131 patients. Classification was according to the extent of cutaneous involvement, type I (28%): sclerosis as far as the wrist-joint; type II (65%): sclerosis beyond the wrist-joint; type III (7%): sclerosis beginning to affect the trunk. In addition, they were subdivided into those with or without signs of systemic inflammation. The sex ratio was 3.5 females to 1 male. In half of the patients the disease manifested itself between the age of 30 and 50 years, with a clear peak about the 40th year. Serological evidence of inflammatory and immunological phenomena was present in nearly 50%, predominantly in those with type II or III. Oesophageal involvement was present in 84%, of the lung in 56% of all patients. ECG and echocardiographic changes were demonstrated in 34% of patients, while liver, intestines and kidneys were only rarely affected. The degree of organ involvement increased from type I to type III. Signs of systemic inflammation were predominantly associated with a clinically severe course.
根据收集到的131例患者的数据，对进行性系统性硬化症的病程进行了分析。根据皮肤受累程度进行分类，I型（28%）：硬化累及至腕关节；II型（65%）：硬化超出腕关节；III型（7%）：硬化开始累及躯干。此外，将患者细分为有或无全身炎症体征的两类。男女比例为1:3.5。半数患者在30至50岁之间发病，在40岁左右有一个明显的发病高峰。近50%的患者存在炎症和免疫现象的血清学证据，主要见于II型或III型患者。84%的患者有食管受累，56%的患者有肺部受累。34%的患者有心电图和超声心动图改变，而肝脏、肠道和肾脏受累较少。器官受累程度从I型到III型逐渐增加。全身炎症体征主要与临床严重病程相关。
