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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...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2023
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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. |
format | Online Article Text |
id | pubmed-10616785 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
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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