Wegener's granulomatosis. Clinical features and outcome in 13 patients

Arch Intern Med. 1983 Mar;143(3):476-9. doi: 10.1001/archinte.143.3.476.


Thirteen patients with Wegener's granulomatosis were seen over 10.5 years. The clinical features resembled those in previously reported series, except for the increased frequency of inflammatory arthritis, which was a prominent early feature in ten patients (77%). Four (31%) of the 13 had fulminant vasculitis and died before receiving an adequate course of cytotoxic drug therapy. Two of these four had a pulmonary-renal syndrome that mimicked Goodpasture's syndrome. All of the remaining nine patients (69%) achieved an initial remission with cytotoxic agents (azathioprine or cyclophosphamide), but four died in less than one year with no evidence of vasculitis at autopsy. The 56% survival rate to one year in these nine patients contrasts with an 86% to 100% survival in other series. Chronic renal failure was a prominent sequela in those who survived one year.

MeSH terms

  • Adolescent
  • Aged
  • Antibiotics, Antineoplastic / therapeutic use*
  • Azathioprine / therapeutic use
  • Cyclophosphamide / therapeutic use
  • Female
  • Granulomatosis with Polyangiitis / complications
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / physiopathology*
  • Humans
  • Male
  • Middle Aged
  • Prednisone / therapeutic use
  • Prognosis


  • Antibiotics, Antineoplastic
  • Cyclophosphamide
  • Azathioprine
  • Prednisone