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

Am J Kidney Dis. 2015 Oct;66(4):613-20. doi: 10.1053/j.ajkd.2015.03.025. Epub 2015 May 12.

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).

Study design: Cohort study.

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.

Predictor: 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").

Outcomes: Incidence, causes of death, and survival.

Measurements: 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).

Limitations: 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.

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).

Publication types

  • Evaluation Study

MeSH terms

  • Adult
  • Case-Control Studies
  • Cause of Death
  • Disease Progression
  • Disease-Free Survival
  • Europe
  • Female
  • Granulomatosis with Polyangiitis / diagnosis
  • Granulomatosis with Polyangiitis / mortality*
  • Granulomatosis with Polyangiitis / therapy*
  • Humans
  • Kaplan-Meier Estimate
  • Kidney Failure, Chronic / diagnosis
  • Kidney Failure, Chronic / mortality*
  • Kidney Failure, Chronic / therapy
  • Kidney Function Tests
  • Kidney Transplantation / methods
  • Kidney Transplantation / mortality
  • Male
  • Microscopic Polyangiitis / diagnosis
  • Microscopic Polyangiitis / mortality*
  • Microscopic Polyangiitis / therapy*
  • Middle Aged
  • Prognosis
  • Proportional Hazards Models
  • Registries*
  • Renal Dialysis / methods
  • Renal Dialysis / mortality
  • Severity of Illness Index
  • Societies, Medical
  • Survival Rate