Incidence of remission and relapse of proteinuria, end-stage kidney disease, mortality, and major outcomes in primary nephrotic syndrome: the Japan Nephrotic Syndrome Cohort Study (JNSCS)

Ryohei Yamamoto, Enyu Imai, Shoichi Maruyama, Hitoshi Yokoyama, Hitoshi Sugiyama, Kosaku Nitta, Tatsuo Tsukamoto, Shunya Uchida, Asami Takeda, Toshinobu Sato, Takashi Wada, Hiroki Hayashi, Yasuhiro Akai, Megumu Fukunaga, Kazuhiko Tsuruya, Kosuke Masutani, Tsuneo Konta, Tatsuya Shoji, Takeyuki Hiramatsu, Shunsuke GotoHirofumi Tamai, Saori Nishio, Arimasa Shirasaki, Kojiro Nagai, Kunihiro Yamagata, Hajime Hasegawa, Hideo Yasuda, Shizunori Ichida, Tomohiko Naruse, Tomoya Nishino, Hiroshi Sobajima, Satoshi Tanaka, Toshiyuki Akahori, Takafumi Ito, Yoshio Terada, Ritsuko Katafuchi, Shouichi Fujimoto, Hirokazu Okada, Eiji Ishimura, Junichiro J. Kazama, Keiju Hiromura, Tetsushi Mimura, Satoshi Suzuki, Yosuke Saka, Tadashi Sofue, Yusuke Suzuki, Yugo Shibagaki, Kiyoki Kitagawa, Kunio Morozumi, Yoshiro Fujita, Makoto Mizutani, Takashi Shigematsu, Naoki Kashihara, Hiroshi Sato, Seiichi Matsuo, Ichiei Narita, Yoshitaka Isaka

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Abstract

Background: Despite recent advances in immunosuppressive therapy for patients with primary nephrotic syndrome, its effectiveness and safety have not been fully studied in recent nationwide real-world clinical data in Japan. Methods: A 5-year cohort study, the Japan Nephrotic Syndrome Cohort Study, enrolled 374 patients with primary nephrotic syndrome in 55 hospitals in Japan, including 155, 148, 38, and 33 patients with minimal change disease (MCD), membranous nephropathy (MN), focal segmental glomerulosclerosis (FSGS), and other glomerulonephritides, respectively. The incidence rates of remission and relapse of proteinuria, 50% and 100% increases in serum creatinine, end-stage kidney disease (ESKD), all-cause mortality, and other major adverse outcomes were compared among glomerulonephritides using the Log-rank test. Incidence of hospitalization for infection, the most common cause of mortality, was compared using a multivariable-adjusted Cox proportional hazard model. Results: Immunosuppressive therapy was administered in 339 (90.6%) patients. The cumulative probabilities of complete remission within 3 years of the baseline visit was ≥ 0.75 in patients with MCD, MN, and FSGS (0.95, 0.77, and 0.79, respectively). Diabetes was the most common adverse events associated with immunosuppressive therapy (incidence rate, 71.0 per 1000 person-years). All-cause mortality (15.6 per 1000 person-years), mainly infection-related mortality (47.8%), was more common than ESKD (8.9 per 1000 person-years), especially in patients with MCD and MN. MCD was significantly associated with hospitalization for infection than MN. Conclusions: Patients with MCD and MN had a higher mortality, especially infection-related mortality, than ESKD. Nephrologists should pay more attention to infections in patients with primary nephrotic syndrome.

Original languageEnglish
Pages (from-to)526-540
Number of pages15
JournalClinical and Experimental Nephrology
Volume24
Issue number6
DOIs
Publication statusPublished - 01-06-2020

All Science Journal Classification (ASJC) codes

  • Physiology
  • Nephrology
  • Physiology (medical)

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    Yamamoto, R., Imai, E., Maruyama, S., Yokoyama, H., Sugiyama, H., Nitta, K., Tsukamoto, T., Uchida, S., Takeda, A., Sato, T., Wada, T., Hayashi, H., Akai, Y., Fukunaga, M., Tsuruya, K., Masutani, K., Konta, T., Shoji, T., Hiramatsu, T., ... Isaka, Y. (2020). Incidence of remission and relapse of proteinuria, end-stage kidney disease, mortality, and major outcomes in primary nephrotic syndrome: the Japan Nephrotic Syndrome Cohort Study (JNSCS). Clinical and Experimental Nephrology, 24(6), 526-540. https://doi.org/10.1007/s10157-020-01864-1