Rituximab for refractory focal segmental glomerulosclerosis

被引:105
作者
Nakayama, Makiko [1 ]
Kamei, Koichi [1 ]
Nozu, Kandai [2 ]
Matsuoka, Kentaro [3 ]
Nakagawa, Atsuko [3 ]
Sako, Mayumi [1 ]
Iijima, Kazumoto [1 ]
机构
[1] Natl Ctr Child Hlth & Dev, Childrens Natl Med Ctr, Dept Nephrol, Setagaya Ku, Tokyo 1578535, Japan
[2] Kobe Univ, Grad Sch Med, Dept Pediat, Kobe, Hyogo, Japan
[3] Natl Ctr Child Hlth & Dev, Dept Pathol, Tokyo, Japan
关键词
steroid-resistant nephrotic syndrome; focal segmental glomerulosclerosis; plasmapheresis; rituximab; B cells;
D O I
10.1007/s00467-007-0640-x
中图分类号
R72 [儿科学];
学科分类号
100202 [儿科学];
摘要
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.
引用
收藏
页码:481 / 485
页数:5
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