METHODS: RESULTS: The distribution according to ANCAs was 14
p-ANCA (58%), 5
c-ANCA (21%) and 5
ANCA-negative (21%) pauci-immune renal
vasculitis.
Pulmonary renal syndrome (PRS) was observed in 10 patients at the onset of
vasculitis.
Corticosteroids and daily
cyclophosphamide were administered to 18 patients, and one patient had intravenous
cyclophosphamide. Five patients received isolated
corticosteroid therapy. Early reduction in
cyclophosphamide dosage was required in five patients due to leucopaenia. Mean follow-up after first dialysis was 89 ± 66 months (range 2-208). Twenty patients were included in haemodialysis (HD), and four patients were included in
peritoneal dialysis (PD). At the end of the study, nine patients had received a cadaveric kidney transplant (KT). Relapses rate after the onset of dialysis was 0.03 episode/patient/year. PRS-associated relapses after beginning dialysis were observed in four patients. Main
therapy in relapses was also
corticosteroids and
cyclophosphamide. Survival rates for year 1, 2 and 5 was 91%, 91% and 85%, respectively. Overall mortality at the end of the study was 31.8%. Five patients died in the PRS group, but only one death was associated with progressive
pulmonary fibrosis. Higher mortality was observed in PRS
vasculitis present at the onset of RRT (50% vs 16.7%, P = NS). Better outcome in patients who received a
renal transplantation was observed (88.8% vs 53.8%, P = NS). Conclusions. Despite a low number of patients in this series,
pauci-immune vasculitis prognosis under dialysis seems equal to other causes of
chronic kidney disease. This study observed a low rate of relapses after beginning dialysis. Poor prognosis is related to severe complications at the beginning of RRT. Today,
kidney transplantation is an important therapeutic option for these patients.