[Clinical aspects of progressive systemic scleroderma (PSS). Multicenter studies of 194 patients]

Patients from five German Departments of Dermatology (Düsseldorf, Erlangen, Frankfurt am Main, Hamburg and Munich) affected with progressive systemic scleroderma (PSS) were classified and examined. The results of the clinical investigations are presented. In order to guarantee a uniform classification of all patients, the patients were divided into three groups according to the distribution of the affected skin: type I consisted of those with acrosclerosis distal to the wrist, type II had scleroderma extending along the wrist in a proximal direction, and type III had diffuse scleroderma beginning on the trunk. Altogether, 194 patients with PSS were investigated, and the following distribution was found: type I, 32%; type II, 60%; type III, 7%; 1% of the patients could not be classified. The distribution according to the patients' sex and age was in good agreement with published reference data. The incidence, significance, and localization of the major symptoms were investigated. The Raynaud symptom could be identified as being the main clinical symptom in 90% of the patients. Joint involvements (10%-73% depending in the applied parameters), dysphagia (51%), and rest dyspnea (30%) contributed to the main internal symptoms. The extensive clinical, chemical, and immunological results are summarized. In 80% of the cases, high ANA titers could be detected, but these were not correlated to the type of disease.