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

• Published in: International urology and nephrology 2018-07, Vol.50 (7), p.1311-1318
• Main Authors: Bjørneklett, Rune, Solbakken, Vilde, Bostad, Leif, Fismen, Anne-Siri
• Format: Article
• Language: English
• Subjects: Adult; Aged; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - epidemiology; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - pathology; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis - therapy; Antineutrophil cytoplasmic antibodies; Biopsy; Biopsy, Needle; Cohort analysis; Cohort Studies; Disease Progression; End-stage renal disease; Female; Females; Glomerulonephritis; Histology; History, Medieval; Humans; Immunohistochemistry; Incidence; Kidney diseases; Kidney Failure, Chronic - epidemiology; Kidney Failure, Chronic - pathology; Kidney Failure, Chronic - therapy; Male; Males; Medicine; Medicine & Public Health; Middle Aged; Nephrology; Nephrology - Original Paper; Norway - epidemiology; Prognosis; Proportional Hazards Models; Registries; Serology; Severity of Illness Index; Sex Distribution; Survival Analysis; Urology; Vasculitis
• ISSN: 0301-1623; 1573-2584
• DOI: 10.1007/s11255-018-1888-8

Purpose 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. Methods 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. Results 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