[Progressive systemic scleroderma. Clinical spectrum and prognostic parameters in 131 patients].
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.
D000328:Adult; D001145:Arrhythmias, Cardiac / Q000209:etiology; D003286:Contracture / Q000209:etiology; D003967:Diarrhea / Q000209:etiology; D004576:Electromyography; D005260:Female; D005385:Fingers; D005764:Gastroesophageal Reflux / Q000209:etiology; D006333:Heart Failure / Q000209:etiology; D006801:Humans; D006977:Hypertension, Renal / Q000209:etiology; D000860:Hypoxia / Q000209:etiology; D008103:Liver Cirrhosis / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010490:Pericardial Effusion / Q000209:etiology; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000145:classification / Q000150:complications / Q000473:pathology*; D012737:Sex Factors
Progressive systemische Sklerodermie. Klinisches Spektrum und prognostische Parameter von 131 Patienten.