Briedigkeit L, Ulmer M, Rheinhold-Keller E, Göbel U, Natusch R, Gross W L
I. Medizinische Klinik, Klinikums Berlin-Buch.
Z Gesamte Inn Med. 1993 Apr;48(4):183-9.
Treatment and outcome of 111 patients who fell ill with Wegener's granulomatosis (WG) between 1966 and 1990 were analysed retrospectively. The mean observation time after diagnosis had been 47 (1-288) months. At the time of diagnosis 37 patients suffered from locoregional symptoms only, whereas the disease was generalised in 74 patients. Two regimes of treatment were differentiated: "conventional" treatment, i.e. daily application of cyclophosphamide/prednisolone (FAUCI scheme) or azathioprine/prednisolone or prednisolone alone, and "stage-adapted" treatment, characterised by change of different treatments (e.g. cyclophosphamide pulse therapy, cotrimoxazole) according to the extent and activity of disease. In patients who received stage-adapted treatment, relapses occurred significantly more frequently than in conventionally treated patients. On the other hand, lethal outcome was much more frequent in conventionally treated patients.