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Rituximab for refractory focal segmental glomerulosclerosis

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Abstract

We present the cases of two children with steroid-resistant nephrotic syndrome (SRNS) who were treated with rituximab (anti-CD20 monoclonal antibody). Both were resistant to conventional therapy, and renal biopsy showed focal segmental glomerulosclerosis (FSGS). Combination therapy with methylprednisolone pulse therapy and plasmapheresis was the only way to decrease proteinuria. However, the patients suffered severe reactions to steroid treatment. We therefore treated them with rituximab in a single dose of 375 mg/m2, which resulted in the rapid clearing of circulating CD19-positive B cells. One month after rituximab treatment, both achieved partial remission; one patient has maintained complete remission for 8 months, and the other relapsed 8 months after the first rituximab treatment with the recovery of peripheral B-cell counts and received a second rituximab treatment. She achieved complete remission 5 months after the second course and has maintained the remission for 2 months. We conclude that rituximab may be an effective treatment for refractory SRNS with FSGS.

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Authors and Affiliations

  1. Department of Nephrology, National Children’s Medical Center, National Center for Child Health and Development, 10-1, Okura 2 chome, Setagaya-ku, Tokyo, 157-8535, Japan

    Makiko Nakayama, Koichi Kamei, Mayumi Sako & Kazumoto Iijima

  2. Department of Pediatrics, Kobe University Graduate School of Medicine, Kobe, Japan

    Kandai Nozu

  3. Department of Pathology, National Center for Child Health and Development, Tokyo, Japan

    Kentaro Matsuoka & Atsuko Nakagawa

Authors
  1. Makiko Nakayama
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  2. Koichi Kamei
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  3. Kandai Nozu
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  4. Kentaro Matsuoka
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  5. Atsuko Nakagawa
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  6. Mayumi Sako
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  7. Kazumoto Iijima
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Correspondence to Kazumoto Iijima.

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Nakayama, M., Kamei, K., Nozu, K. et al. Rituximab for refractory focal segmental glomerulosclerosis. Pediatr Nephrol 23, 481–485 (2008). https://doi.org/10.1007/s00467-007-0640-x

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  • DOI: https://doi.org/10.1007/s00467-007-0640-x

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