World Journal of Pediatrics

, Volume 10, Issue 1, pp 59–63

Efficacy of rituximab therapy in children with refractory nephrotic syndrome: a prospective observational study in Shanghai

  • Li Sun
  • Hong Xu
  • Qian Shen
  • Qi Cao
  • Jia Rao
  • Hai-Mei Liu
  • Xiao-Yan Fang
  • Li-Jun Zhou
Original article

DOI: 10.1007/s12519-014-0453-5

Cite this article as:
Sun, L., Xu, H., Shen, Q. et al. World J Pediatr (2014) 10: 59. doi:10.1007/s12519-014-0453-5

Abstract

Background

Idiopathic nephrotic syndrome is the most common glomerular disease in children. This study was undertaken to observe the efficacy and side-effects of rituximab (RTX) in treating children with different types of refractory primary nephrotic syndrome.

Methods

Twelve patients with steroid dependent nephrotic syndrome (SDNS), frequently relapsing nephritic syndrome (FRNS), and steroid resistant nephrotic syndrome (SRNS) were enrolled in our study. There were obvious drug side-effects, and proteinuria remained difficult to control. RTX was administered at a dose of 375 mg/m2 body surface area, once or twice weekly.

Results

The male to female ratio was 3:1, and the onset age was 1.6–8.9 years. There were 9 patients with steroid sensitive nephrotic syndrome (SDNS or FRNS), and 3 patients with SRNS. There were 7 patients with minimal change disease (MCD), 3 patients with focal segmental glomerular sclerosis (FSGS), 1 with focal proliferative glomerulonephritis, and 1 without renal biopsy. The total effective treatment rate of RTX was 91.67%, and for 77.78% of the patients, steroid dosage could be reduced. Six months before and after RTX infusion, the mean steroid dosage was significantly decreased (P=0.014) and the recurrence number was significantly reduced (P<0.001). The results were better in MCD patients than in FSGS patients (P=0.045). There was no significant difference between FRNS/SDNS and SRNS patients (P=0.175). During RTX administration, 3 patients developed skin rashes, 1 developed hypotension, and 1 developed a fever. One patient experienced a persistent decrease in serum immunoglobulin level but without serious infection.

Conclusion

RTX was effective in the treatment of refractory nephrotic syndrome, and it could significantly reduce the use of steroid and immunosuppressants.

Key words

refractory nephritic syndromerituximab

Copyright information

© Children's Hospital, Zhejiang University School of Medicine and Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  • Li Sun
    • 1
  • Hong Xu
    • 1
  • Qian Shen
    • 1
  • Qi Cao
    • 1
  • Jia Rao
    • 1
  • Hai-Mei Liu
    • 1
  • Xiao-Yan Fang
    • 1
  • Li-Jun Zhou
    • 1
  1. 1.Department of Nephrology and RheumatologyChildren’s Hospital of Fudan UniversityShanghaiChina