This study describes the incidence and outcomes of European patients requiring renal
replacement therapy (RRT) for kidney failure due to antineutrophil cytoplasmic antibody
(ANCA)-associated vasculitis (AAV).
Setting & Participants
12 renal registries providing individual RRT patient data to the European Renal Association–European
Dialysis and Transplant Association (ERA-EDTA) Registry in 1993-2012 participated.
Cause of primary kidney disease: AAV (ie, granulomatosis with polyangiitis [Wegener]
and microscopic polyangiitis) versus 3 separate matched control groups without AAV:
(1) primary glomerulonephritis, (2) diabetes mellitus, and (3) disease other than
diabetes mellitus as the cause of primary kidney disease, including glomerulonephritis
Incidence, causes of death, and survival.
ERA-EDTA primary renal disease codes.
2,511 patients with AAV (1,755, granulomatosis with polyangiitis; 756, microscopic
polyangiitis) were identified, representing an incidence of 1.05 per million population
(pmp) for granulomatosis with polyangiitis (predominating in Northern Europe) and
0.45 pmp for microscopic polyangiitis (prevailing in Southern Europe). Kidney transplantation
was performed in 558 (22.2%) patients with vasculitis. The 10-year probability for
survival on RRT after day 91 was 32.5% (95% CI, 29.9%-35.1%) in patients with vasculitis.
Survival on RRT after day 91 did not differ between AAV and matched nondiabetes patients.
Patient and transplant survival after kidney transplantation, adjusted for time period
and country, was better in AAV than in matched nondiabetes patients (HRs of 0.81 [95%
CI, 0.67-0.99] and 0.82 [95% CI, 0.69-0.96], respectively).
No data for extrarenal manifestations, treatment, and relapses.
Geographical differences in the incidence of RRT for kidney failure due to granulomatosis
with polyangiitis and microscopic polyangiitis copied their distribution in the general
population. Overall survival on RRT after day 91 for patients with AAV was similar
to that for patients with nondiabetes diagnoses. Our results suggest that patients
with AAV are suitable candidates for kidney transplantation with favorable survival