title: [Progressive systemic sclerosis - long-term treatment with azathioprin (author's transl)].
abstract: The study presents the results of an Azathioprin long-term therapy in 19 of 60 patients with progressive systemic sclerosis (PSS). Average treatment was 47 months (6 to 114 months). The patients received 2-2,5 Azathioprin/kg bodyweight daily. In 16 cases no further progression of PSS was noted. One patient showed minor deterioration. In particular no further deterioration in the lung and kidney manifestations were found. Two patients died. A female patient showed signs of osteomyelofibrosis after being treated for 70 months. She died 3,5 years after Azathioprin had been discontinued. The second patient died of right heart failure after recurrent pulmonary emboli. On the whole treatment with Azathioprin over a long period of time seems in most cases to hold the progression of the disease. The unfavourable prognosis can therefore be much improved.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000032:analysis; D001379:Azathioprine / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D055728:Primary Myelofibrosis / Q000139:chemically induced; D011379:Prognosis; D011655:Pulmonary Embolism / Q000150:complications; D011660:Pulmonary Heart Disease / Q000209:etiology; D012595:Scleroderma, Systemic / Q000188:drug therapy*
chemical_list: D000974:Antibodies, Antinuclear; D001379:Azathioprine
vernacular_title: Langzeitbehandlung der progressiven Systemsklerose mit Azathioprin.