title: Improved prognosis in Norwegian patients with glomerulonephritis associated with anti-neutrophil cytoplasmic antibodies.
abstract: BACKGROUND
  Glomerulonephritis associated with anti-neutrophil cytoplasmic antibodies (ANCA) is associated with increased mortality and a high risk of end-stage renal disease (ESRD). Here, we investigated whether the prognosis has improved over the last 25 years.
  
  
  METHODS
  Patients were identified in the Norwegian Kidney Biopsy Registry. We included all patients with pauci-immune crescentic glomerulonephritis and a positive ANCA test from 1988 to 2012. Deaths and ESRD in the cohort were identified through record linkage with the Norwegian Population Registry (deaths) and the Norwegian Renal Registry (ESRD). Outcomes of patients diagnosed in 1988-2002 were compared with outcomes of patients diagnosed in 2003-12.
  
  
  RESULTS
  A cohort of 455 patients with ANCA-associated glomerulonephritis was identified. The mean follow-up was 6.0 years (range, 0.0-23.4). During the study period, 165 (36%) patients died and 124 (27%) progressed to ESRD. Compared with patients diagnosed in 1988-2002, those diagnosed in 2003-12 had higher mean initial estimated glomerular filtration rates (37 versus 27 mL/min/1.73 m(2)) and lower risk of ESRD (1-year risk: 13 versus 19%; 10-year risk: 26 versus 37%). The composite endpoint, ESRD or death within 0-1 year after diagnosis, was reduced from 34 to 25%. In patients over 60 years old, 1-year mortality fell from 33 to 20%.
  
  
  CONCLUSIONS
  In Norwegian patients with ANCA-associated glomerulonephritis, prognosis was significantly better in 2003-12 compared with 1988-2002. This improvement was probably partly due to a shorter diagnostic delay, and better therapeutic management in older patients.
authors.affiliation: Renal Research Group, Department of Clinical Medicine, University of Bergen, Bergen, Norway.
authors.forename: Sanjeevan
authors.initials: S
authors.lastname: Sriskandarajah
authors.affiliation: Department of Nephrology, St Olavs University Hospital, Trondheim, Norway Faculty of Medicine, Norwegian University of Science and Technology, Trondheim, Norway.
authors.forename: Knut
authors.initials: K
authors.lastname: Aasarød
authors.affiliation: Department of Medicine, Haukeland University Hospital, Bergen, Norway Department of Clinical Science, University of Bergen, Bergen, Norway.
authors.forename: Steinar
authors.initials: S
authors.lastname: Skrede
authors.affiliation: Renal Research Group, Department of Clinical Medicine, University of Bergen, Bergen, Norway Department of Medicine, Haukeland University Hospital, Bergen, Norway.
authors.forename: Thomas
authors.initials: T
authors.lastname: Knoop
authors.affiliation: Norwegian Renal Registry, Section of Nephrology, Department of Transplant Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
authors.forename: Anna Varberg
authors.initials: AV
authors.lastname: Reisæter
authors.affiliation: Renal Research Group, Department of Clinical Medicine, University of Bergen, Bergen, Norway Department of Medicine, Haukeland University Hospital, Bergen, Norway.
authors.forename: Rune
authors.initials: R
authors.lastname: Bjørneklett
mesh_terms: D000328:Adult; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000175:diagnosis* / Q000401:mortality; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality; D006801:Humans; D008297:Male; D008875:Middle Aged; D009664:Norway; D011379:Prognosis; D015996:Survival Rate; D055815:Young Adult
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: ANCA-associated glomerulonephritis; diagnostic delay; end-stage renal disease; mortality; prognosis