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Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a nationwide 1989–2015 survey

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Rapidly progressive glomerulonephritis (RPGN) can progress to end-stage kidney disease within a short period. This study is a continuation of the chronological nationwide survey conducted by the Japan-RPGN working group.


We examined a total of 2793 RPGN cases registered during four periods (1989–1998, 1999–2001, 2002–2008, 2009–2011) plus 1386 cases in 2012–2015. As potential prognostic determinants, we investigated the onset period, the clinical severity (CS) grade [classified according to age, serum creatinine (sCr) and C-reactive protein levels, and presence/absence of lung lesions], and causative disease.


The cumulative overall RPGN patient survival at 24 months kept improving over the five periods (72.0%, 72.9%, 77.7%, 83.0%, 84.9%, p < 0.001 for trend). The cumulative renal survival also improved in the latest period (68.7%, 75.4%, 76.7%, 73.4%, 78.2%, p < 0.001 for trend). The CS grade was well stratified to predict both life and renal prognoses. Anti-glomerular basement membrane disease (aGBMD)-RPGN had a poorer renal prognosis than other diseases. In anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV-RPGN, accounting for > 70% of the overall RPGN), the prognostic results were similar to that for overall RPGN. There was a much better renal prognosis for the latest period under the condition of sCr < 3 mg/dL (the 24-month cumulative renal survival: 97.9%), but not for sCr ≥ 3 mg/dL (61.5%).


In overall RPGN as well as AAV-RPGN, both life and renal prognoses tended to improve, but the favorable renal result was substantially limited to mild cases. There was no improvement of the renal prognosis in aGBMD-RPGN.

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We thank the following members of the RPGN Working Group of the Research Committee of Intractable Renal Disease of the Ministry of Health, Labour, and Welfare (MHLW) of Japan: Drs. Shinya Kaname, Tatsuo Tsukumoto, Eri Muso, Kousaku Nitta, Takashi Wada, Toshinobu Sato, Masaki Kobayashi, Junichiro Nakata, Takashi Yokoo, Yukio Yuzawa, Kouichi Asahi, Kosuke Masuya, Shouichi Fujimoto, Shinji Hirawa, Tomo Suzuki, Wako Yumura, Takashi Ito, Kazuhiko Tsuruya, Masayuki Iwano, Ken-ei Sada, Yasunori Iwata, Ryohei Kaseda, Seiichi Matsuo, Naoto Yamaguchi, and Akio Koyama. We also thank the nationwide investigators and institutions that participated in this survey and Mss. Yuko Sudo and. Keiko Fujioka for their guidance in manuscript preparation. This work was supported by grants from the Research Committee of Intractable Renal Disease of the MHLW of Japan.

The protocol of the present study was approved by the Ethics Committee of the University of Tsukuba Hospital (No. R1-020). The study was conducted according to the Declaration of Helsinki.

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Correspondence to Kunihiro Yamagata.

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All the authors have declared no competing interest.

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The data for this survey obtained from the patients’ medical records were anonymized for personal information so that the patients cannot be identified. Informed consent was obtained from all individual patients included in the study. Each participating facility disclosed the purpose of the survey, method, survey period, survey contents, and privacy protection, and they provided all registered patients an opportunity to opt out.

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Kaneko, S., Yamagata, K., Usui, J. et al. Epidemiology and temporal changes in the prognosis of rapidly progressive glomerulonephritis in Japan: a nationwide 1989–2015 survey. Clin Exp Nephrol 26, 234–246 (2022).

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