Respiratory failure in Wegener's granulomatosis: response to pulse intravenous methylprednisolone and cyclophosphamide

Abstract

We describe a patient with respiratory failure due to Wegener's granulomatosis (WG) who obtained a dramatic and sustained response to pulse intravenous methylprednisolone and cyclophosphamide. Discussion includes a review of the literature with respect to the relative merits of daily oral cyclophosphamide versus pulse intravenous cyclophosphamide in WG and other vasculitides. While the combination of daily oral steroids and daily oral cyclophosphamide dramatically improves the outcome of WG, this therapy is not without serious side effects. Pulse therapy may be equally as effective and may lessen the frequency of serious side effects.