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PubMed · 9548575

Wegener's granulomatosis.

L E Harman·C E Margo
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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.

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Antibodies, Antineutrophil CytoplasmicBiopsyDiagnosis, DifferentialEye DiseasesFluorescent Antibody Technique, IndirectGlucocorticoidsGranulomatosis with PolyangiitisHumansImmunosuppressive AgentsSeverity of Illness IndexJournal ArticleReview

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L E Harman, C E Margo. Wegener's granulomatosis.. https://doi.org/10.1016/s0039-6257(97)00133-1

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