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

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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.

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Acknowledgments

We express our thanks to the doctors who participated in this observational study. We also express our gratitude to Ms. Yuko Sudo, Ms. Keiko Fujioka, and Ms. Michiko Yokoyama for manuscript preparation, as well as to the members of the RPGN Study Group of Progressive Renal Disease from the Ministry of Health, Labor, and Welfare of Japan as follows; Dr. Y Taguma, Dr. S Kaname, Dr. S Horikoshi, Dr. T Hosoya, Dr. T Kawamura, Dr. Y Yuzawa, Dr. T Watanabe, Dr. T Saitoh, Dr. S Fujimoto, Dr. S Hirawa, Dr. K Kimura, Dr. W Yumura, Dr. T Itoh, Dr. K Tabei, Dr. O Inaguma, Dr. M Ogura, Dr. S Yasunaga, Dr. K Tsuruya, Dr. N Nakagawa, Dr. M Yoshida, Dr. S Maruyama, and Dr. K Sada. This study was supported in part by a Grant-in-Aid for Progressive Renal Diseases Research, Research on intractable disease, from the Ministry of Health, Labor, and Welfare of Japan.

Conflict of interest

The authors declare no conflicts of interest.

Author information

Authors and Affiliations

  1. Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1-1-1 Ten-oudai, Tsukuba, Ibaraki, 305-8575, Japan

    Kunihiro Yamagata, Joichi Usui, Chie Saito, Naoto Yamaguchi, Kouichi Hirayama, Kaori Mase, Masaki Kobayashi & Akio Koyama

  2. Steering Committee for the Japanese RPGN Study Group of Progressive Renal Disease, Tsukuba, Japan

    Kunihiro Yamagata, Hitoshi Sugiyama, Kosaku Nitta, Takashi Wada, Eri Muso, Yoshihiro Arimura, Hirofumi Makino & Seiichi Matsuo

Authors
  1. Kunihiro Yamagata
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  2. Joichi Usui
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  3. Chie Saito
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  4. Naoto Yamaguchi
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  5. Kouichi Hirayama
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  6. Kaori Mase
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  7. Masaki Kobayashi
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  8. Akio Koyama
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  9. Hitoshi Sugiyama
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  10. Kosaku Nitta
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  11. Takashi Wada
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  12. Eri Muso
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  13. Yoshihiro Arimura
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  14. Hirofumi Makino
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  15. Seiichi Matsuo
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Corresponding author

Correspondence to Kunihiro Yamagata.

Additional information

On behalf of the Japanese RPGN Study Group of Progressive Renal Disease.

Members of The Japanese RPGN Study Group of Progressive Renal Disease are Kunihiro Yamagata, Masaki Kobayashi, Akio Koyama, Hitoshi Sugiyama, Kosaku Nitta, Takashi Wada, Eri Muso, Yoshihiro Arimura, Hirofumi Makino, and Seiichi Matsuo.

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Yamagata, K., Usui, J., Saito, C. et al. ANCA-associated systemic vasculitis in Japan: clinical features and prognostic changes. Clin Exp Nephrol 16, 580–588 (2012). https://doi.org/10.1007/s10157-012-0598-2

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  • DOI: https://doi.org/10.1007/s10157-012-0598-2

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