ANCA-associated systemic vasculitis in Japan: Clinical features and prognostic changes

Kunihiro Yamagata, Joichi Usui, Chie Saito, Naoto Yamaguchi, Kouichi Hirayama, Kaori Mase, Masaki Kobayashi, Akio Koyama, Hitoshi Sugiyama, Kosaku Nitta, Takashi Wada, Eri Muso, Yoshihiro Arimura, Hirofumi Makino, Seiichi Matsuo

Research output: Contribution to journalArticlepeer-review

49 Citations (Scopus)


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.

Original languageEnglish
Pages (from-to)580-588
Number of pages9
JournalClinical and Experimental Nephrology
Issue number4
Publication statusPublished - Aug 2012
Externally publishedYes


  • Anti-neutrophil cytoplasmic auto-antibody (ANCA)
  • Immunosuppression
  • Prognosis changes
  • Rapidly progressive glomerulonephritis (RPGN)
  • Vasculitis

ASJC Scopus subject areas

  • Physiology
  • Nephrology
  • Physiology (medical)


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