title: Wegener's granulomatosis.
abstract: 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.
authors.affiliation: Department of Ophthalmology, University of South Florida, College of Medicine, Tampa, USA.
authors.forename: L E
authors.initials: LE
authors.lastname: Harman
authors.forename: C E
authors.initials: CE
authors.lastname: Margo
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis; D001706:Biopsy; D003937:Diagnosis, Differential; D005128:Eye Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D019084:Fluorescent Antibody Technique, Indirect; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D012720:Severity of Illness Index
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007166:Immunosuppressive Agents