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Master Thesis

Efficacité et sécurité du Rituximab dans le syndrome néphrotique idiopathique cortico-dépendant de l’enfant à court et long terme. Analyse rétrospective monocentrique de 33 cas

Abstract : Context: Idiopathic childhood steroid-dependent nephrotic syndrome (SDNS) remains a therapeutic challenge, since remission is maintained thanks to steroid and immunosuppressants with significant drug toxicity. The aim of this study was to investigate the short and long-term efficacy and safety of Rituximab (RTX), an anti-CD20 monoclonal antibody, in idiopathic childhood SDNS. Methods: We retrospectively collected clinical and biological data of 33 children with idiopathic SDNS treated with RTX, leading to a minimal B-cell depletion of 15 months. Results: At the end of a median follow-up after B-cell recovery of 27 months [20–45], the complete remission rate (no relapse after B-cell recovery) reached 73%. In addition, we observed a significant decrease in cumulative treatment duration (steroid and immunosuppressants) after RTX (p<0,0001). Main median and long-term side effects included infections (n=18, 55%), with one fulminant viral myocarditis leading to a heart transplant surgery. Eight patients (24%) had low IgG levels during B-cell depletion, and 12 were concerned after B-cell recovery (36%), including 6 children without any previous history of hypogammaglobulinemia. Interestingly, patients who underwent supplementation with intravenous polyvalent immunoglobulins (IgIV) had longer relapse-free duration (40 ± 7,2 versus 21 ± 2,5 months, p=0,0045). Conclusions: RTX is an effective therapeutic option for children with idiopathic SDNS, leading to a long-lasting remission, but requires a close monitoring. Further studies are necessary to investigate the association with IgIV and their immunomodulating effects.
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Master Thesis
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https://dumas.ccsd.cnrs.fr/dumas-01130918
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Submitted on : Thursday, March 12, 2015 - 3:21:51 PM
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  • HAL Id : dumas-01130918, version 1

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Aurore Ventre Surun. Efficacité et sécurité du Rituximab dans le syndrome néphrotique idiopathique cortico-dépendant de l’enfant à court et long terme. Analyse rétrospective monocentrique de 33 cas. Médecine humaine et pathologie. 2014. ⟨dumas-01130918⟩

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