Wegener's granulomatosis

Surv Ophthalmol. 1998 Mar-Apr;42(5):458-80. doi: 10.1016/s0039-6257(97)00133-1.


Clinical manifestations of Wegener's granulomatosis are nonspecific and indistinguishable from a variety of neoplastic, infectious, and inflammatory diseases. Ophthalmic disease is the presenting feature in nearly one sixth of patients with Wegener's granulomatosis and will ultimately develop in a majority. The discovery of antineutrophil cytoplasmic antibodies, particularly antiproteinase-3, has changed the clinical approach to evaluating patients suspected of having Wegener's granulomatosis. These antibodies are distinguished from other related autoantibodies because they produce a coarse granular pattern of cytoplasmic staining on indirect immunofluorescence with ethanol-fixed neutrophils. Treatment of Wegener's granulomatosis with oral cyclophosphamide and corticosteroids has decreased morbidity and improved survival, but side effects from long-term immunosuppressive therapy are common and sometimes serious. The effectiveness of trimethoprim-sulfamethoxazole in decreasing the number and severity of recurrences of Wegener's granulomatosis is being investigated. It remains to be determined if wide use of trimethoprim-sulfamethoxazole in limited Wegener's granulomatosis could further improve the quality of life for some patients.

Publication types

  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / analysis
  • Biopsy
  • Diagnosis, Differential
  • Eye Diseases / diagnosis*
  • Eye Diseases / drug therapy
  • Eye Diseases / immunology
  • Fluorescent Antibody Technique, Indirect
  • Glucocorticoids / therapeutic use
  • Granulomatosis with Polyangiitis / diagnosis*
  • Granulomatosis with Polyangiitis / drug therapy
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Severity of Illness Index


  • Antibodies, Antineutrophil Cytoplasmic
  • Glucocorticoids
  • Immunosuppressive Agents