Rituximab in children with steroid sensitive nephrotic syndrome: in quest of the optimal regimen

Rituximab has emerged as an effective and important therapy in children with complicated frequently relapsing and steroid-dependent nephrotic syndrome to induce long-term disease remission and avoid steroid toxicities. The optimal rituximab regimen is not totally well defined, and there are many var...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 2021-06, Vol.36 (6), p.1397-1405
Main Authors: Chan, Eugene Yu-hin, Tullus, Kjell
Format: Article
Language:English
Subjects:
Autoimmune diseases
Child
Child, Preschool
Children
Clinical outcomes
Disease
Drug dosages
Drug therapy
Educational Review
Ethnicity
Glucocorticoids
Humans
Hypogammaglobulinemia
Immunosuppression
Immunotherapy
Kidney diseases
Lupus
Lymphoma
Medicine
Medicine & Public Health
Methods
Monoclonal antibodies
Nephrology
Nephrotic syndrome
Nephrotic Syndrome - drug therapy
Patients
Pediatric research
Pediatrics
Recurrence
Remission
Remission (Medicine)
Rituximab
Rituximab - administration & dosage
Steroids - adverse effects
Steroids - therapeutic use
Treatment Outcome
Urology
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Summary:Rituximab has emerged as an effective and important therapy in children with complicated frequently relapsing and steroid-dependent nephrotic syndrome to induce long-term disease remission and avoid steroid toxicities. The optimal rituximab regimen is not totally well defined, and there are many varying practices worldwide. We will in this review describe how patient factors, rituximab dose, and use of maintenance immunosuppression affect treatment outcomes. Specifically, low-dose rituximab without concomitant immunosuppression is associated with shorter relapse-free duration while other regimens have comparable outcomes. Patients with more severe disease generally have worse response to rituximab. Although rituximab appears to be generally safe, there are growing concerns of chronic hypogammaglobulinemia and impaired immunity especially in young children. Reliable prognostications and biomarkers for guiding subsequent treatments to avoid excessive treatments are yet to be identified. In this review, we will outline the, as we see it, best approach of rituximab in childhood steroid sensitive nephrotic syndrome at the present state of knowledge.
ISSN:0931-041X
1432-198X
DOI:10.1007/s00467-020-04609-0