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Rituximab in refractory nephrotic syndrome

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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.

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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

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Acknowledgments

We thank the International Pediatric Nephrology Association for help in the distribution of questionnaires. We are very grateful to all paediatric nephrologists who contributed to this study by submitting data on their patients: B. Ahmed (Dubai, UAE), L. Al-Khalidi, (Dubai, UAE), A. Amore (Torino, Italy), I. Al Attrach (Abu Dhabi, UAE), J. Baluarte (Philadelphia, USA), D. Ben-Ezer (Orange CA, USA), V. de Barros Bittencourt (Porto Alegre, Brazil), J. Booth (Newcastle upon Tyne, UK), J. Chen (Toronto, Canada), R. Coppo (Torino, Italy), C. Crompton (Perth, Australia), K. Fidan (Ankara, Turkey), A. Gür Güven (Antalya, Turkey), R. Hani Hadidi (Amman, Jordan), E. Harvey (Toronto, Canada), D. Hebert (Toronto, Canada), J. Huang (Beijing, China), S. Hulton (Birmingham, UK), F. Hussain (Nottingham, UK), L. Hyla-Klekot (Chorzow, Poland), B. Kaplan (Philadelphia, USA), L. Peruzzi (Torino, Italy), V. Pinto (Santiago, Chile), G. Porcellini (Torino, Italy), I. Roberti (New York, USA), E. Simon (Albany, USA), O. Söylemezoğlu (Ankara, Turkey), K. Van Hoeck (Antwerp, Belgium).

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Correspondence to Kjell Tullus.

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Prytuła, A., Iijima, K., Kamei, K. et al. Rituximab in refractory nephrotic syndrome. Pediatr Nephrol 25, 461–468 (2010). https://doi.org/10.1007/s00467-009-1376-6

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  • DOI: https://doi.org/10.1007/s00467-009-1376-6

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