WEGENERS GRANULOMATOSIS

Clinical manifestations of Wegener's granulomatosis are nonspecific an d indistinguishable from a variety of neoplastic, infectious, and infl ammatory diseases. Ophthalmic disease is the presenting feature in nea rly one sixth of patients with Wegener's granulomatosis and will ultim ately develop in a majority. The discovery of antineutrophil cytoplasm ic antibodies, particularly antiproteinase-3, has changed the clinical approach to evaluating patients suspected of having Wegener's granulo matosis. These antibodies are distinguished from other related autoant ibodies 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 si de effects from long-term immunosuppressive therapy are common and som etimes serious. The effectiveness of trimethoprim-sulfamethoxazole in decreasing the number and severity of recurrences of Wegener's granulo matosis 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. (C) 1998 by Elsevier Science Inc. All rights reserved.).