A case report of wegener granulomatosis treated only with corticosteroids for 30 years

Abstract

Background: Wegener granulomatosis is a systemic vasculitis classically described as involving the upper and lower respiratory tracts together with glomerulonephritis. Its poor prognosis was marginally improved with the use of corticosteroids, and long-term remission was not achieved until the introduction of cytotoxic agents. Generalized or systemic Wegener granulomatosis has a worse prognosis than those limited to the respiratory tracts.

Objective: To report the case of an individual who we suspect has a 30-year history of Wegener granulomatosis treated only with prednisone.

Methods: A literature search was performed with PubMed using the keywords Wegener granulomatosis, survival, prognosis, and treatment.

Results: Our patient is alive 32 years after her initial symptoms of vasculitis. She has been taking daily prednisone only for the majority of this time. Our clinical diagnosis of Wegener granulomatosis is based on the history and very high antineutrophil cytoplasmic antibody with the cytoplasmic pattern.

Conclusions: There may be variants of generalized Wegener granulomatosis that survive with less aggressive treatment.