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A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity

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Abstract

Background

The etiology, prevalence, and prognosis of rapidly progressive glomerulonephritis (RPGN) including renal vasculitis vary among races and periods.

Method

To improve the prognosis of Japanese RPGN patients, we conducted a nationwide survey of RPGN in the nephrology departments of 351 tertiary hospitals, and found 1772 patients with RPGN (Group A: diagnosed between 1989 and 1998, 884 cases; Group B: diagnosed between 1999 and 2001, 321 cases; and Group C: diagnosed between 2002 and 2007, 567 cases). ANCA subclasses, renal biopsy findings, treatment, outcome and cause of death were recorded.

Result

The most frequent primary disease was renal-limited vasculitis (RLV) (42.1%); the second was microscopic polyangiitis (MPA) (19.4%); the third was anti-GBM-associated RPGN (6.1%). MPO-ANCA was positive in 88.1% of RLV patients and 91.8% of MPA patients. The proportion of primary renal diseases of RPGN was constant during those periods. The most frequent cause of death was infectious complications. The serum creatinine at presentation and the initial dose of oral prednisolone decreased significantly in Groups B and C compared to Group A. However, both patient and renal survival rates improved significantly in Groups B and C (survival rate after six months in Group A: 79.2%, Group B: 80.1%, and Group C: 86.1%. Six-month renal survival in Group A: 73.3%, Group B: 81.3%, and Group C: 81.8%).

Conclusion

Early diagnosis was the most important factor for improving the prognosis of RPGN patients. To avoid early death due to opportunistic infection in older patients, a milder immunosuppressive treatment such as an initial oral prednisolone dose reduction with or without immunosuppressant is recommended.

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Acknowledgment

We express our thanks to the doctors who participated with this observational study (listed in Table 9). The authors also express their gratitude to Ms. Yuko Sudo, Ms Keiko Fujioka, and Ms. Sanae Hasegawa for manuscript preparation, as well as to Dr. Hideto Takahashi for statistical analysis, and to Dr. Kouichi Hirayama, Dr. Kaori Mase, Dr. Naoto Yamaguchi, Dr. Chie Saitoh, Dr. Joichi Usui and Dr. Masaki Kobayashi for valuable discussion and preparation of data. This study was supported in part by a grant in relation to Progressive Renal Disease from the Ministry of Health, Labor and Welfare Research Project for Specially Selected Disease.

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Authors and Affiliations

  1. Ibaraki Prefuctural University, Ami, Japan

    Akio Koyama

  2. Department of Nephrology, Institute of Clinical Medicine, Graduate School of Comprehensive Human Sciences, University of Tsukuba, 1-1-1 Ten-oudai, Tsukuba, Ibaraki, 305-8575, Japan

    Kunihiro Yamagata

  3. Department of Medicine and Clinical Science, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan

    Hirofumi Makino

  4. First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan

    Yoshihiro Arimura

  5. Department of Laboratory Medicine, Graduate School of Medical Science, Kanazawa University, Kanazawa, Japan

    Takashi Wada

  6. Department of Medicine, Kidney Center, Tokyo Women’s Medical University, Tokyo, Japan

    Kosaku Nitta & Hiroshi Nihei

  7. Department of Nephrology and Dialysis, The Tazuke Kofukai Medical Research Institute, Kitano Hospital, Osaka, Japan

    Eri Muso

  8. Department of Nephrology, Sendai Shakaihoken Hospital, Sendai, Japan

    Yoshio Taguma

  9. Department of Pathology, Shinsyu University, Matsumoto, Japan

    Hidekazu Shigematsu

  10. Division of Nephrology and Metabolism, Department of Internal Medicine, Tokai University, School of Medicine, Kanagawa, Japan

    Hideto Sakai

  11. Division of Nephrology, Department of Internal Medicine, Juntendo University School of Medicine, Tokyo, Japan

    Yasuhiko Tomino

  12. Department of Nephrology, Nagoya University Graduate School of Medicine, Nagoya, Japan

    Seiichi Matsuo

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  1. Akio Koyama
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  2. Kunihiro Yamagata
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  3. Hirofumi Makino
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  4. Yoshihiro Arimura
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  5. Takashi Wada
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  6. Kosaku Nitta
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  7. Hiroshi Nihei
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  8. Eri Muso
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  9. Yoshio Taguma
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  10. Hidekazu Shigematsu
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  11. Hideto Sakai
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  12. Yasuhiko Tomino
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  13. Seiichi Matsuo
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Consortia

Japan RPGN Registry Group

Corresponding author

Correspondence to Kunihiro Yamagata.

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Koyama, A., Yamagata, K., Makino, H. et al. A nationwide survey of rapidly progressive glomerulonephritis in Japan: etiology, prognosis and treatment diversity. Clin Exp Nephrol 13, 633–650 (2009). https://doi.org/10.1007/s10157-009-0201-7

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  • DOI: https://doi.org/10.1007/s10157-009-0201-7

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