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Wegener's granulomatosis: clinical features and prognosis in 37 patients
Author(s) -
Romas E.,
Kennedy J. T.,
Murphy B. F.,
Niall J. F.,
'Apice A. J. F.
Publication year - 1993
Publication title -
australian and new zealand journal of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 0004-8291
DOI - 10.1111/j.1445-5994.1993.tb01812.x
Subject(s) - medicine , cyclophosphamide , case fatality rate , surgery , sepsis , incidence (geometry) , vasculitis , mortality rate , stage (stratigraphy) , disease , epidemiology , chemotherapy , paleontology , physics , optics , biology
:Thirty‐seven patients (21 female, 16 male) with Wegener's granulomatosis (WG)were reviewed. Patients were followed for a mean six years after diagnosis; 14 were followed for more than seven years. The clinical features were similar to those in previous studies. In this series, only 13 patients (35%) had renal disease at presentation and the cumulative incidence of renal involvement was 51%. Thirty‐one patients received treatment which included cyclophosphamide (CP). The case fatality rate of the six patients not treated with CP was 83% (five deaths). By contrast, all CP treated patients improved and 21 (68%) had complete remissions. Nine (29%) were in complete remission for a mean 4.9 years after discontinuing all treatment. Two were disease free for over ten years. The actuarial probability of survival for these patients was 97% at one year and 71% at ten years. Only three CP treated patients (10%) progressed to end‐stage renal disease. The case fatality rate was 26% (eight patients) and sepsis was the cause of death in five. Fourteen patients (45%) treated with CP had at least one relapse of vasculitis and seven (23%) had multiple (two or more) relapses. These data indicate that CP is effective in inducing remissions and prolonging survival in patients with WG; however, relapses are frequent. (Aust NZ J Med 1993; 23: 168–175.)