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Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system. Relapse and incomplete recovery from relapse are common in NMOSD. Most patients with NMOSD have IgG to aquaporin-4 (AQP4-IgG). New biological agents for AQP4-IgG-seropositive NMOSD, such as sat...

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Autores principales: Li, Xiaojing, Wu, Wenlin, Zeng, Yiru, Wu, Wenxiao, Hou, Chi, Zhu, Haixia, Liao, Yinting, Tian, Yang, Chen, Zongzong, Peng, Bingwei, Chen, Wen-Xiong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10616785/
https://www.ncbi.nlm.nih.gov/pubmed/37915570
http://dx.doi.org/10.3389/fimmu.2023.1257955
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author Li, Xiaojing
Wu, Wenlin
Zeng, Yiru
Wu, Wenxiao
Hou, Chi
Zhu, Haixia
Liao, Yinting
Tian, Yang
Chen, Zongzong
Peng, Bingwei
Chen, Wen-Xiong
author_facet Li, Xiaojing
Wu, Wenlin
Zeng, Yiru
Wu, Wenxiao
Hou, Chi
Zhu, Haixia
Liao, Yinting
Tian, Yang
Chen, Zongzong
Peng, Bingwei
Chen, Wen-Xiong
author_sort Li, Xiaojing
collection PubMed
description Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system. Relapse and incomplete recovery from relapse are common in NMOSD. Most patients with NMOSD have IgG to aquaporin-4 (AQP4-IgG). New biological agents for AQP4-IgG-seropositive NMOSD, such as satralizumab, have become available for maintenance therapy. Satralizumab is an anti-interleukin-6 receptor monoclonal antibody. To date, few studies have evaluated satralizumab as an add-on treatment in pediatric NMOSD patients. Here, we report an 11-year-old girl with NMOSD who frequently relapsed under long-term treatment, including oral prednisone, rituximab, mycophenolate mofetil (MMF), and maintenance intravenous immunoglobulin treatment even with B-cell depletion. For the poor treatment response and to improve the efficacy of relapse prevention further, the patient received satralizumab treatment as an add-on therapy to MMF plus oral prednisone, with a dose of 120 mg administered subcutaneously at weeks 0, 2, and 4 and every 4 weeks after that. After initiating satralizumab, the patient remained relapse-free for 14 months at the last follow-up. Satralizumab might be effective and safe as an add-on treatment in refractory pediatric AQP4-IgG-seropositive NMOSD under B-cell depletion.
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spelling pubmed-106167852023-11-01 Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report Li, Xiaojing Wu, Wenlin Zeng, Yiru Wu, Wenxiao Hou, Chi Zhu, Haixia Liao, Yinting Tian, Yang Chen, Zongzong Peng, Bingwei Chen, Wen-Xiong Front Immunol Immunology Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disease of the central nervous system. Relapse and incomplete recovery from relapse are common in NMOSD. Most patients with NMOSD have IgG to aquaporin-4 (AQP4-IgG). New biological agents for AQP4-IgG-seropositive NMOSD, such as satralizumab, have become available for maintenance therapy. Satralizumab is an anti-interleukin-6 receptor monoclonal antibody. To date, few studies have evaluated satralizumab as an add-on treatment in pediatric NMOSD patients. Here, we report an 11-year-old girl with NMOSD who frequently relapsed under long-term treatment, including oral prednisone, rituximab, mycophenolate mofetil (MMF), and maintenance intravenous immunoglobulin treatment even with B-cell depletion. For the poor treatment response and to improve the efficacy of relapse prevention further, the patient received satralizumab treatment as an add-on therapy to MMF plus oral prednisone, with a dose of 120 mg administered subcutaneously at weeks 0, 2, and 4 and every 4 weeks after that. After initiating satralizumab, the patient remained relapse-free for 14 months at the last follow-up. Satralizumab might be effective and safe as an add-on treatment in refractory pediatric AQP4-IgG-seropositive NMOSD under B-cell depletion. Frontiers Media S.A. 2023-10-17 /pmc/articles/PMC10616785/ /pubmed/37915570 http://dx.doi.org/10.3389/fimmu.2023.1257955 Text en Copyright © 2023 Li, Wu, Zeng, Wu, Hou, Zhu, Liao, Tian, Chen, Peng and Chen https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Li, Xiaojing
Wu, Wenlin
Zeng, Yiru
Wu, Wenxiao
Hou, Chi
Zhu, Haixia
Liao, Yinting
Tian, Yang
Chen, Zongzong
Peng, Bingwei
Chen, Wen-Xiong
Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report
title Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report
title_full Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report
title_fullStr Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report
title_full_unstemmed Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report
title_short Satralizumab as an add-on treatment in refractory pediatric AQP4-antibody-positive neuromyelitis optica spectrum disorder: a case report
title_sort satralizumab as an add-on treatment in refractory pediatric aqp4-antibody-positive neuromyelitis optica spectrum disorder: a case report
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10616785/
https://www.ncbi.nlm.nih.gov/pubmed/37915570
http://dx.doi.org/10.3389/fimmu.2023.1257955
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