Rituximab for refractory focal segmental glomerulosclerosis

• DOI: 10.1007/s00467-007-0640-x
• Publication Date: 2007-10-31T08:04:48Z
• Authors: Makiko Nakayama; Koichi Kamei; Kandai Nozu; Kentaro Matsuoka; Atsuko Nakagawa; Mayumi Sako; Kazumoto Iijima
• Keywords: Glomerulosclerosis, Focal Segmental; Remission Induction; Antibodies, Monoclonal; 3. Good health; Antibodies, Monoclonal, Murine-Derived; 03 medical and health sciences; 0302 clinical medicine; Humans; Immunologic Factors; Female; Child; Rituximab

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/m(2), 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.