title: Clinical outcomes of Japanese MPO-ANCA-related nephritis: significance of initial renal death for survival.
abstract: OBJECTIVE
  Anti-myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA)- related nephritis constitutes 60% of rapidly progressive glomerulonephritis (RPGN) in Japan. The reported 1-year survival rate is over 80%, however, the long-term prognosis remains unknown. We therefore investigated the prognosis and factors affecting the clinical course of patients.
  
  
  METHODS
  We retrospectively investigated 74 patients (female, n=42; median age, 73.0 years) with MPO-ANCA-related nephritis. The patients were admitted to Fukushima Medical University and two affiliated hospitals between 2000 and 2010.
  
  
  RESULTS
  Median estimated GFR (eGFR) was 12.1 mL/min/1.73 m2 at admission. The Birmingham Vasculitis Activity Score (BVAS version 3: max 63 points) at diagnosis and at 4 weeks after start of treatment were 15.0 and 5.0, respectively. Twenty-three patients (31%) died during a median observation period of 30.5 months. Sixteen patients (22%) presented with end-stage renal disease (ESRD) at the initial phase, and needed regular dialysis therapy. Multivariate Cox proportional hazards model analysis revealed that renal death at the initial phase was a significant risk factor for all-cause death (Hazard ratio, 5.72; 95% confidence interval, 2.49-13.09; p<0.001). Furthermore, BVAS>6, evaluated 4 weeks after start of treatment, is an independent risk factor for ESRD and patient survival.
  
  
  CONCLUSIONS
  This is the first investigation to demonstrate clinical features focusing on MPO-ANCA-related nephritis. Renal death at the initial phase of treatment is a powerful risk factor for all-cause death in patients with MPO-ANCA-related nephritis. Patients at high risk of death and ESRD could be stratified according to BVAS.
authors.affiliation: Department of Nephrology, Fukushima Medical University School of Medicine, Japan. ag9t2wja@fmu.ac.jp
authors.forename: Kimio
authors.initials: K
authors.lastname: Watanabe
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Tani
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kimura
authors.forename: Kenichi
authors.initials: K
authors.lastname: Tanaka
authors.forename: Yoshimitsu
authors.initials: Y
authors.lastname: Hayashi
authors.forename: Koichi
authors.initials: K
authors.lastname: Asahi
authors.forename: Keiji
authors.initials: K
authors.lastname: Sato
authors.forename: Mamoru
authors.initials: M
authors.lastname: Sato
authors.forename: Masato
authors.initials: M
authors.lastname: Matsushima
authors.forename: Masaaki
authors.initials: M
authors.lastname: Nakayama
authors.forename: Tsuyoshi
authors.initials: T
authors.lastname: Watanabe
mesh_terms: D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000209:etiology / Q000276:immunology / Q000401:mortality; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology*; D005260:Female; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000150:complications / Q000276:immunology* / Q000401:mortality* / Q000503:physiopathology; D006801:Humans; D007564:Japan / Q000453:epidemiology; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000276:immunology / Q000401:mortality; D008297:Male; D009195:Peroxidase / Q000276:immunology*; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D009195:Peroxidase