Pediatric Nephrology

, Volume 26, Issue 5, pp 733–737

Rituximab for refractory cases of childhood nephrotic syndrome

  • Jameela A. Kari
  • Salah M. El-Morshedy
  • Sherif El-Desoky
  • Hammad O. Alshaya
  • Khawla A. Rahim
  • Burhan M. Edrees
Original Article

DOI: 10.1007/s00467-011-1778-0

Cite this article as:
Kari, J.A., El-Morshedy, S.M., El-Desoky, S. et al. Pediatr Nephrol (2011) 26: 733. doi:10.1007/s00467-011-1778-0

Abstract

Rituximab has been used over the last decade as a rescue therapy for refractory cases of nephrotic syndrome (NS). Here we report the use of rituximab in four children with idiopathic steroid-resistant nephrotic syndrome (SRNS) with various histological backgrounds (two cases with focal segmental glomerulosclerosis, one case with IgM nephropathy, and one case with minimal change disease), who failed to respond to other immunsuppressions. Their median age (range) was 10 (8–11) years. NPHS2 genetic mutation was negative in all of them. All patients received a single dose of rituximab (375 mg/m2) and achieved complete B cell depletion as CD19 was <1% for 3 months following rituximab infusion. Only one patient achieved non-sustained remission as he relapsed after 4 months despite zero CD19 level. Patients received no further doses of rituximab as B cell was depleted in the peripheral circulation. We conclude that a single dose of rituximab was not effective in inducing sustained remission in children with idiopathic SRNS, despite complete B cell depletion in the peripheral circulation. Further doses might be indicated to deplete non-circulating B cells.

Keywords

Rituximab Idiopathic steroid-resistant nephrotic syndrome (SRNS) CD19 B cell depletion 

Copyright information

© IPNA 2011

Authors and Affiliations

  • Jameela A. Kari
    • 1
  • Salah M. El-Morshedy
    • 1
  • Sherif El-Desoky
    • 1
  • Hammad O. Alshaya
    • 2
  • Khawla A. Rahim
    • 2
  • Burhan M. Edrees
    • 3
  1. 1.Department of Pediatrics and Princess Al-Jawharah Center of Excellence in Research for Hereditary DiseasesKing AbdulAziz UniversityJeddahSaudi Arabia
  2. 2.Department of PediatricsKing Fahad Medical CityRiyadhSaudi Arabia
  3. 3.Faculty of MedicineUmm Al-Qura UniversityMakkahSaudi Arabia

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