title: 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.
abstract: BACKGROUND
  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).
  
  
  METHODS
  Cohort study.
  
  
  METHODS
  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.
  
  
  METHODS
  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 (termed "nondiabetes").
  
  
  RESULTS
  Incidence, causes of death, and survival.
  
  
  METHODS
  ERA-EDTA primary renal disease codes.
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  No data for extrarenal manifestations, treatment, and relapses.
  
  
  CONCLUSIONS
  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 outcomes.
authors.affiliation: Department of Nephrology, General University Hospital and First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic. Electronic address: zd.hruskova@gmail.com.
authors.forename: Zdenka
authors.initials: Z
authors.lastname: Hruskova
authors.affiliation: ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Vianda S
authors.initials: VS
authors.lastname: Stel
authors.affiliation: Department of Medicine, University of Cambridge, Cambridge, United Kingdom.
authors.forename: David
authors.initials: D
authors.lastname: Jayne
authors.affiliation: Department of Renal Medicine, St Olav's University Hospital, Trondheim, Norway.
authors.forename: Knut
authors.initials: K
authors.lastname: Aasarød
authors.affiliation: Department of Nephrology, Dialysis and Hypertension, AZ Nikolaas, Sint-Niklaas, Belgium.
authors.forename: Johan
authors.initials: J
authors.lastname: De Meester
authors.affiliation: Division of Nephrology, Department of Medicine, Helsinki University Hospital, Helsinki, Finland.
authors.forename: Agneta
authors.initials: A
authors.lastname: Ekstrand
authors.affiliation: Clinical Division of Nephrology, Department of Internal Medicine, Medical University of Graz, Graz, Austria.
authors.forename: Kathrin
authors.initials: K
authors.lastname: Eller
authors.affiliation: Department of Nephrology, Herlev Hospital, University of Copenhagen, Copenhagen, Denmark.
authors.forename: James G
authors.initials: JG
authors.lastname: Heaf
authors.affiliation: Division of Nephrology, Radboud University Medical Center, Nijmegen, the Netherlands.
authors.forename: Andries
authors.initials: A
authors.lastname: Hoitsma
authors.affiliation: Centre for Public Health Research (CSISP-FISABIO), Valencia, Spain.
authors.forename: Carmen
authors.initials: C
authors.lastname: Martos Jimenéz
authors.affiliation: Department of Medicine, University of Calgary, Calgary, Canada; Department of Community Health Sciences, University of Calgary, Calgary, Canada.
authors.forename: Pietro
authors.initials: P
authors.lastname: Ravani
authors.affiliation: Division of Nephrology, Department of Medicine, University Hospital, Würzburg, Germany.
authors.forename: Christoph
authors.initials: C
authors.lastname: Wanner
authors.affiliation: Department of Nephrology, General University Hospital and First Faculty of Medicine, Charles University in Prague, Prague, Czech Republic.
authors.forename: Vladimir
authors.initials: V
authors.lastname: Tesar
authors.affiliation: ERA-EDTA Registry, Department of Medical Informatics, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Kitty J
authors.initials: KJ
authors.lastname: Jager
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D002423:Cause of Death; D018450:Disease Progression; D018572:Disease-Free Survival; D005060:Europe; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D006801:Humans; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D007677:Kidney Function Tests; D016030:Kidney Transplantation / Q000379:methods / Q000401:mortality; D008297:Male; D055953:Microscopic Polyangiitis / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy*; D008875:Middle Aged; D011379:Prognosis; D016016:Proportional Hazards Models; D012042:Registries; D006435:Renal Dialysis / Q000379:methods / Q000401:mortality; D012720:Severity of Illness Index; D012955:Societies, Medical; D015996:Survival Rate
keywords: ANCA-associated vasculitis (AAV); Antineutrophil cytoplasmic antibody (ANCA); dialysis; end-stage renal disease (ESRD); granulomatosis with polyangiitis (GPA) [Wegener]; kidney transplantation; microscopic polyangiitis (MPA); outcomes; renal replacement therapy (RRT)