• Am. J. Kidney Dis. · Oct 2015

    Characteristics and Outcomes of Granulomatosis With Polyangiitis (Wegener) and Microscopic Polyangiitis Requiring Renal Replacement Therapy: Results From the European Renal Association-European Dialysis and Transplant Association Registry.

    • Zdenka Hruskova, Vianda S Stel, David Jayne, Knut Aasarød, Johan De Meester, Agneta Ekstrand, Kathrin Eller, James G Heaf, Andries Hoitsma, Carmen Martos Jimenéz, Pietro Ravani, Christoph Wanner, Vladimir Tesar, and Kitty J Jager.
    • Department of Nephrology, General University Hospital and First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic. Electronic address: zd.hruskova@gmail.com.
    • Am. J. Kidney Dis. 2015 Oct 1; 66 (4): 613-20.

    BackgroundThis 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).Study DesignCohort study.Setting & Participants12 renal registries providing individual RRT patient data to the European Renal Association-European Dialysis and Transplant Association (ERA-EDTA) Registry in 1993-2012 participated.PredictorCause 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 (termed "nondiabetes").OutcomesIncidence, causes of death, and survival.MeasurementsERA-EDTA primary renal disease codes.Results2,511 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).LimitationsNo data for extrarenal manifestations, treatment, and relapses.ConclusionsGeographical 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 outcomes.Copyright © 2015 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

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