title: Rituximab for refractory focal segmental glomerulosclerosis.
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/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.
authors.affiliation: 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.
authors.forename: Makiko
authors.initials: M
authors.lastname: Nakayama
authors.forename: Koichi
authors.initials: K
authors.lastname: Kamei
authors.forename: Kandai
authors.initials: K
authors.lastname: Nozu
authors.forename: Kentaro
authors.initials: K
authors.lastname: Matsuoka
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakagawa
authors.forename: Mayumi
authors.initials: M
authors.lastname: Sako
authors.forename: Kazumoto
authors.initials: K
authors.lastname: Iijima
mesh_terms: D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D002648:Child; D005260:Female; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D012074:Remission Induction; D000069283:Rituximab
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab