Survival and vasculitis activity in patients with end-stage renal disease due to Wegener's granulomatosis.
BACKGROUND
In patients with end-stage renal disease (ESRD) due to Wegener's granulomatosis, a decrease in vasculitis activity after the development of ESRD, as described in other autoimmune diseases, has been postulated. However, up to now no data in a larger group of patients with Wegener's granulomatosis on chronic dialysis have been available.


METHODS
We retrospectively analysed the clinical course of 35 patients with Wegener's granulomatosis and ESRD during chronic dialysis treatment. Diagnosis was based on clinical manifestation, antineutrophil cytoplasmic antibodies and/or histology.


RESULTS
During a mean follow-up of 43 months (5-113 months), six patients died, three related to treatment toxicity. The patient survival rates (according to Kaplan-Meier calculation) were 93% after 2 years and 79% after 5 years. Twenty-nine relapses of Wegener's granulomatosis occurred in 17 patients (relapse rate 0.24/patient/year); 2/3 of the relapses were seen during treatment with steroids, 1/6 during cyclophosphamide therapy. The relapses were not related to the dialysis membrane used. Remission or partial remission could be achieved in 93% of the relapses.


CONCLUSIONS
The survival of patients on chronic dialysis treatment due to Wegener's granulomatosis was comparable to that of other patient groups with ESRD. The relapse rate was not different from that of non-dialysed patients with Wegener's granulomatosis, and this finding underlines the need for a therapeutic strategy to maintain long-term remission in dialysis-dependent patients, too.
Department of Nephrology, Medical School Hannover, Germany.
M
M
Haubitz
K M
KM
Koch
R
R
Brunkhorst
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D005858:Germany / Q000453:epidemiology; D014890:Granulomatosis with Polyangiitis / Q000150:complications*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D007688:Kidneys, Artificial; D008297:Male; D008567:Membranes, Artificial; D008875:Middle Aged; D011379:Prognosis; D012008:Recurrence; D006435:Renal Dialysis; D012189:Retrospective Studies; D015996:Survival Rate; D014657:Vasculitis / Q000209:etiology*
D007166:Immunosuppressive Agents; D008567:Membranes, Artificial; D003520:Cyclophosphamide