title: ANCA-associated systemic vasculitis in Japan: clinical features and prognostic changes.
abstract: BACKGROUND
  This study was conducted to standardize treatment and determine patient and renal outcome in Japanese anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis/rapidly progressive glomerulonephritis (AAV/RPGN) patients, because the prognosis of AAV/RPGN patients in Japan had been poor compared with that of other countries.
  
  
  METHODS
  The participants in this retrospective cohort study were 824 ANCA-positive RPGN patients, 705 of whom were only myeloperoxidase (MPO)-ANCA positive.
  
  
  RESULTS
  Among the early-years cohort (group A; cases diagnosed between 1988 and 1998), patients frequently died due to opportunistic infection. Therefore, we recommended a reduced dose of prednisolone (oral prednisolone dose <0.8 mg/kg/day) with or without cyclophosphamide for initial treatment of Japanese RPGN patients. After this recommendation, 1-year survival of the patients improved: 75% in group A, 79% in group B (between 1999 and 2002), and 81% in group C (after 2003). During the entire observation period, average serum creatinine level at the start of treatment decreased, and improvement of 1-year renal survival was also found (72% in group A, 83% in group B, and 83% in group C), while the recurrence rate was significantly increased in group C (0.05/patient-year in group A, 0.07/patient-year in group B, and 0.13/patient-year in group C).
  
  
  CONCLUSIONS
  Oral prednisolone dose <0.8 mg/kg/day with or without cyclophosphamide as an initial treatment could improve patient survival in older Japanese AAV/RPGN patients. However, maintenance treatment avoiding relapse should be established to improve renal outcomes.
authors.affiliation: Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Ten-oudai, Tsukuba, Ibaraki 305-8575, Japan. kidney@md.tsukuba.ac.jp
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Yamagata
authors.forename: Joichi
authors.initials: J
authors.lastname: Usui
authors.forename: Chie
authors.initials: C
authors.lastname: Saito
authors.forename: Naoto
authors.initials: N
authors.lastname: Yamaguchi
authors.forename: Kouichi
authors.initials: K
authors.lastname: Hirayama
authors.forename: Kaori
authors.initials: K
authors.lastname: Mase
authors.forename: Masaki
authors.initials: M
authors.lastname: Kobayashi
authors.forename: Akio
authors.initials: A
authors.lastname: Koyama
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sugiyama
authors.forename: Kosaku
authors.initials: K
authors.lastname: Nitta
authors.forename: Takashi
authors.initials: T
authors.lastname: Wada
authors.forename: Eri
authors.initials: E
authors.lastname: Muso
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Arimura
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Makino
authors.forename: Seiichi
authors.initials: S
authors.lastname: Matsuo
mesh_terms: D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000175:diagnosis* / Q000188:drug therapy* / Q000453:epidemiology; D015331:Cohort Studies; D003404:Creatinine / Q000097:blood; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use*; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome
chemical_list: D003520:Cyclophosphamide; D011239:Prednisolone; D003404:Creatinine