A Study of Wegener's Granulomatosis Followed over a Long Term

Abstract

We retrospectively investigated the relationship between prognosis and patient's background, presence of renal disease, therapeutic regimen, and side effects of therapies in four cases of Wegener's granulomatosis followed for more than 20 years after diagnosis. As for prognosis, two had a complete remission, one had a complete remission but relapsed, and one didn't have a complete remission and eventually died. The patient who died had a limited form of Wegener's granulomatosis at the first visit, and fot the intitial eight years had a good response to azathioprine and predonisolone. When the lesion extended to a generalized form of Wegener's granulomatosis, azathioprine was replaced by cyclophosphamide. But the patient died of renal failure and infection 4 years after the administration of cyclophosphamide. The two completely remitted cases had limited forms and were given cyclophosphamide soon after the diagnosis without relapse. Early administration of cyclophosphamide may prevent the extension to renal involvement.