Pediatric Nephrology

, Volume 25, Issue 3, pp 461–468

Rituximab in refractory nephrotic syndrome

  • Agnieszka Prytuła
  • Kazumoto Iijima
  • Koichi Kamei
  • Denis Geary
  • Errol Gottlich
  • Abdul Majeed
  • Mark Taylor
  • Stephen D. Marks
  • Shamir Tuchman
  • Roberta Camilla
  • Milos Ognjanovic
  • Guido Filler
  • Graham Smith
  • Kjell Tullus
Original Article

DOI: 10.1007/s00467-009-1376-6

Cite this article as:
Prytuła, A., Iijima, K., Kamei, K. et al. Pediatr Nephrol (2010) 25: 461. doi:10.1007/s00467-009-1376-6

Abstract

The aim of this study was to establish the efficacy and safety of rituximab in refractory nephrotic syndrome (NS). Members of the International Paediatric Nephrology Association were asked to retrospectively fill in a questionnaire with details on the use of rituximab in their centres. We divided the data into three groups: group 1, patients with steroid-dependent and frequently relapsing NS; group 2, with steroid-resistant NS; group 3, with post-transplant recurrence of NS. Seventy questionnaires from 25 centres described the outcome of 28, 27 and 15 patients in groups 1, 2 and 3, respectively. Of these, 82% of patients in group 1, 44% of patients in group 2 and 60% of patients in group 3 had a good initial response. Side effects were observed in 27% of the patients, and these were mostly acute reactions. We present a large multicentre series of children with refractory NS. Children in group 1 showed the best response. The good initial response in group 3 can be biased by the accompanying treatments that were administered at the same time as rituximab. Controlled prospective trials are required to establish the value of rituximab in idiopathic NS.

Keywords

PediatricNephrotic syndromeRemissionRenal transplantation

Abbreviations

ACE-I

angiotensin converting enzyme inhibitors

CI

calcineurin inhibitors

FRNS

frequently relapsing nephrotic syndrome

FSGS

focal and segmental glomerulosclerosis

MCNS

minimal change nephrotic syndrome

MMF

mycophenolate mofetil

NS

nephrotic syndrome

PML

progressive multifocal leukoencephalopathy

PTLD

post-transplant lymphoproliferative disorders

SDNS

steroid-dependent nephrotic syndrome

SLE

systemic lupus erythematosus

SRNS

steroid-resistant nephrotic syndrome

Copyright information

© IPNA 2009

Authors and Affiliations

  • Agnieszka Prytuła
    • 1
  • Kazumoto Iijima
    • 2
    • 3
  • Koichi Kamei
    • 3
  • Denis Geary
    • 4
  • Errol Gottlich
    • 5
  • Abdul Majeed
    • 6
  • Mark Taylor
    • 7
  • Stephen D. Marks
    • 1
  • Shamir Tuchman
    • 8
  • Roberta Camilla
    • 9
  • Milos Ognjanovic
    • 10
  • Guido Filler
    • 11
  • Graham Smith
    • 12
  • Kjell Tullus
    • 1
  1. 1.Department of Paediatric NephrologyGreat Ormond Street Hospital–NHS TrustLondonUK
  2. 2.Division of Child Health and Development, Department of PediatricsKobe University Graduate School of MedicineKobeJapan
  3. 3.Department of NephrologyNational Center for Child Health and DevelopmentTokyoJapan
  4. 4.Division of NephrologyThe Hospital for Sick ChildrenTorontoCanada
  5. 5.Morningside Children’s Kidney Treatment CentreSandtonSouth Africa
  6. 6.Paediatric Nephrology UnitDubai HospitalDubaiUnited Arab Emirates
  7. 7.Birmingham Children’s HospitalBirminghamUK
  8. 8.Division of Pediatric NephrologyThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  9. 9.Nephrology, Dialysis and TransplantationRegina Margherita Children’s HospitalTorinoItaly
  10. 10.Newcastle Children and Young People’s Kidney TeamRoyal Victoria InfirmaryNewcastle upon TyneUK
  11. 11.Children’s Hospital, London Health Science Centre, Department of PaediatricsUniversity of Western OntarioLondonCanada
  12. 12.Children’s Kidney CentreUniversity Hospital of WalesCardiffUK