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Survey of rituximab treatment for childhood-onset refractory nephrotic syndrome

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Abstract

Background

Rituximab (RTX) is a promising option for treating childhood-onset steroid-dependent (SDNS), frequently relapsing (FRNS), and steroid-resistant (SRNS) nephrotic syndrome.

Methods

We retrospectively surveyed RTX treatment for these conditions to evaluate its indications, efficacy and adverse events. Questionnaires were sent to 141 hospitals in Japan.

Results

Seventy-four patients (52 SDNS; 3 FRNS; 19 SRNS) were treated with RTX because of resistance to various immunosuppressive agents. Most patients received a single administration of RTX (85%). Forty-one of 53 SDNS/FRNS (77%) and 5 of 17 SRNS (29%) patients successfully discontinued prednisolone (16 SDNS/FRNS and 6 SRNS achieved their first discontinuation since onset), and 17 out of 53 SDNS/FRNS patients (31%) discontinued cyclosporine. However, 28 of the 53 patients (51%) relapsed. Although immunosuppressive agents did not extend B cell depletion, relapses were significantly less if immunosuppressive agents were continued after RTX (P = 0.006; hazard ratio = 0.2). Among the SRNS patients, complete (n = 6) and partial remission (n = 6) were achieved. No life-threatening adverse events were experienced.

Conclusions

Although this was a multi-center survey where treatment of nephrotic syndrome varied between centers, the steroid-sparing effect of RTX in SDNS/FRNS was excellent. If single administration of RTX is chosen, continuation of immunosuppressive agents is recommended for prevention of relapse.

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Acknowledgements

This work had no financial support. The authors appreciate the doctors who collaborated on our questionnaire: Daishi Hirano, Saitama Children’s Medical Center, Saitama, Japan; Hiroshi Kaito, Kobe University, Kobe, Japan; Tomonori Harada, Yokohama City University Medical Center, Yokohama, Japan; Hiroshi Tanaka, Hirosaki University, Hirosaki, Japan; Toshio Watanabe, Gunma University, Maebashi, Japan; Masaki Shimizu, Kanazawa University, Kanazawa, Japan; Naohiro Wada, Shizouka Children’s Hospital, Shizuoka, Japan; Osamu Uemura, Aichi Children’s Health and Medical Center, Ohbu, Japan; Masashi Nishida, Kyoto Prefectural University of Medicine, Kyoto, Japan; Kenichi Satomura, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka, Japan; Rika Fujimaru, Osaka City General Hospital, Osaka Japan; Ryojiro Tanaka, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan; and Kohei Maekawa, Hyogo College of Medicine, Nishinomiya, Japan.

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Authors and Affiliations

  1. Division of Pediatric Nephrology and Rheumatology, National Center for Child Health and Development, Tokyo, Japan

    Shuichi Ito, Koichi Kamei, Masao Ogura & Tomohiro Udagawa

  2. Division of Nephrology, Saitama Children’s Medical Center, Saitama, Japan

    Shuichiro Fujinaga

  3. Clinical Research Center, National Center for Child Health and Development, Tokyo, Japan

    Mari Saito & Mayumi Sako

  4. Division of Child Health & Development, Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan

    Kazumoto Iijima

  5. Division of Nephrology and Rheumatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan

    Shuichi Ito

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  1. Shuichi Ito
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  2. Koichi Kamei
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Correspondence to Shuichi Ito.

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Ito, S., Kamei, K., Ogura, M. et al. Survey of rituximab treatment for childhood-onset refractory nephrotic syndrome. Pediatr Nephrol 28, 257–264 (2013). https://doi.org/10.1007/s00467-012-2319-1

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  • DOI: https://doi.org/10.1007/s00467-012-2319-1

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