Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years

Ann Intern Med. 1983 Jan;98(1):76-85. doi: 10.7326/0003-4819-98-1-76.

Abstract

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.

Publication types

  • Clinical Trial
  • Review

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Azathioprine / therapeutic use
  • Clinical Trials as Topic
  • Cyclophosphamide / adverse effects
  • Cyclophosphamide / therapeutic use*
  • Drug Administration Schedule
  • Eye Diseases / complications
  • Female
  • Granulomatosis with Polyangiitis / complications
  • Granulomatosis with Polyangiitis / drug therapy*
  • Heart Diseases / complications
  • Humans
  • Joint Diseases / complications
  • Kidney Diseases / complications
  • Lung Diseases / complications
  • Male
  • Middle Aged
  • Muscular Diseases / complications
  • Nervous System Diseases / complications
  • Prednisone / adverse effects
  • Prednisone / therapeutic use*
  • Prospective Studies
  • Respiratory Tract Diseases / complications
  • Skin Diseases / complications

Substances

  • Cyclophosphamide
  • Azathioprine
  • Prednisone