Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years.
Eighty-five patients with Wegener's granulomatosis were studied for 21 years at the National Institutes of Health. Patients were treated with a protocol consisting of cyclophosphamide, 2 mg/kg body weight d, together with prednisone, 1 mg/kg body weight d, followed by conversion of the prednisone to an alternate-day regimen. Complete remissions were achieved in 79 of 85 patients (93%). The mean duration of remission for living patients was 48.2 (+/- 3.6) months. Twenty-three patients are off all therapy for a mean duration of 35.3 (+/- 6.3) months without therapy. This study provides a prospective experience with Wegener's granulomatosis and shows that long-term remissions can be induced and maintained in an extremely high number of patients by the combination of daily cyclophosphamide and alternate-day prednisone therapy.
D000293:Adolescent; D000328:Adult; D000368:Aged; D001379:Azathioprine / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D005128:Eye Diseases / Q000150:complications; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000188:drug therapy*; D006331:Heart Diseases / Q000150:complications; D006801:Humans; D007592:Joint Diseases / Q000150:complications; D007674:Kidney Diseases / Q000150:complications; D008171:Lung Diseases / Q000150:complications; D008297:Male; D008875:Middle Aged; D009135:Muscular Diseases / Q000150:complications; D009422:Nervous System Diseases / Q000150:complications; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use*; D011446:Prospective Studies; D012140:Respiratory Tract Diseases / Q000150:complications; D012871:Skin Diseases / Q000150:complications
D003520:Cyclophosphamide; D001379:Azathioprine; D011241:Prednisone