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Exploring sex-specific differences in the presentation and outcomes of ANCA-associated vasculitis: a nationwide registry-based cohort study



Sex-specific differences in the risk of end-stage renal disease (ESRD) in patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis (ANCA-GN) stratified by histological classification have not been previously investigated.


Patients with biopsy-verified pauci-immune necrotizing GN and positive ANCA serology in the Norwegian Kidney Biopsy Registry between 1991 and 2012 were included. Patients with ESRD during follow-up were identified from the Norwegian Renal Registry. ESRD-free survival stratified by histological classifications was investigated.


We analyzed 358 patients, of whom 87 progressed to ESRD during follow-up. Overall ESRD-free survival at 1 and 5 years in the entire cohort was 81 and 71% in males versus 90 and 80% in females, respectively; 94 and 84% in males versus 98 and 98% in females with focal histology, respectively; 85 and 76% in males versus 89 and 77% in females with mixed histology, respectively; 72 and 58% in males versus 90 and 78% in females with crescentic histology, respectively; and 52 and 46% in males versus 60 and 38% in females with sclerotic histology, respectively. Males had an increased risk of ESRD (adjusted hazard ratio, 2.44 [1.56–3.82]; p < 0.001).


Male sex is associated with increased risk of ESRD across all histological classes of ANCA-GN.

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We thank all nephrologists and pathologists in Norway who reported important data to the Norwegian Kidney Biopsy Registry. We thank Torbjørn Leivestad for linkage of the data from the study cohort with that of the Norwegian Renal Registry. We also thank Editage ( for English language editing.

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Correspondence to Rune Bjørneklett.

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Bjørneklett, R., Solbakken, V., Bostad, L. et al. Exploring sex-specific differences in the presentation and outcomes of ANCA-associated vasculitis: a nationwide registry-based cohort study. Int Urol Nephrol 50, 1311–1318 (2018).

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  • ANCA-associated vasculitis
  • ANCA-associated glomerulonephritis
  • Renal pathology
  • End-stage renal disease