Cargando…

Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder

Neuromyelitis optica spectrum disorder (NMOSD), a severe demyelinating disease, is rare among children. Plasma exchange (PE) is widely used as a salvage therapy for severe and corticosteroid-unresponsive patients with NMOSD. Presently, there are limited studies on the safety and efficacy of PE in ch...

Descripción completa

Detalles Bibliográficos
Autores principales: Li, Zhichao, Wan, Lin, Liu, Xinting, Wang, Jing, Shi, Xiuyu, Zhou, Huanfen, Xu, Quangang, Wei, Shihui, Yang, Guang
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9849793/
https://www.ncbi.nlm.nih.gov/pubmed/36685590
http://dx.doi.org/10.3389/fimmu.2022.1113406
_version_ 1784872030185717760
author Li, Zhichao
Wan, Lin
Liu, Xinting
Wang, Jing
Shi, Xiuyu
Zhou, Huanfen
Xu, Quangang
Wei, Shihui
Yang, Guang
author_facet Li, Zhichao
Wan, Lin
Liu, Xinting
Wang, Jing
Shi, Xiuyu
Zhou, Huanfen
Xu, Quangang
Wei, Shihui
Yang, Guang
author_sort Li, Zhichao
collection PubMed
description Neuromyelitis optica spectrum disorder (NMOSD), a severe demyelinating disease, is rare among children. Plasma exchange (PE) is widely used as a salvage therapy for severe and corticosteroid-unresponsive patients with NMOSD. Presently, there are limited studies on the safety and efficacy of PE in children with NMOSD. Herein, we report the case of six children with NMOSD who received PE along with the outcomes and adverse events. All six children (female, age at onset 4 years 9 months–13 years 2 months) were AQP4-IgG positive and received standard PE using the COM.TEC Cell Separator. The interval between NMOSD onset and PE was 29 days (range 10–98). Only one patient (P3) who received PE 10 days after acute exacerbations exhibited clinical improvement. Her left visual acuity increased from 0.06 to 0.6 (spectacle-corrected visual acuity was 1.0) and her EDSS score decreased from 4 to 3 points. The other five patients had no clinical improvement and no EDSS scores changes after PE. Adverse events included rashes (P1, P3), acute non-occlusive thrombosis of the internal jugular vein (P1), and thrombocytopenia (P2). In conclusion, the timing of PE initiation as a rescue therapy for severe and corticosteroid-unresponsive pediatric AQP4-IgG positive NMOSD may be crucial to treatment efficacy, and early initiation of PE may be associated with a better outcome. Furthermore, PE has the potential risk for clinically significant adverse effects that should be considered before initiating the therapy and should be weighed against potential benefits.
format Online
Article
Text
id pubmed-9849793
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-98497932023-01-20 Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder Li, Zhichao Wan, Lin Liu, Xinting Wang, Jing Shi, Xiuyu Zhou, Huanfen Xu, Quangang Wei, Shihui Yang, Guang Front Immunol Immunology Neuromyelitis optica spectrum disorder (NMOSD), a severe demyelinating disease, is rare among children. Plasma exchange (PE) is widely used as a salvage therapy for severe and corticosteroid-unresponsive patients with NMOSD. Presently, there are limited studies on the safety and efficacy of PE in children with NMOSD. Herein, we report the case of six children with NMOSD who received PE along with the outcomes and adverse events. All six children (female, age at onset 4 years 9 months–13 years 2 months) were AQP4-IgG positive and received standard PE using the COM.TEC Cell Separator. The interval between NMOSD onset and PE was 29 days (range 10–98). Only one patient (P3) who received PE 10 days after acute exacerbations exhibited clinical improvement. Her left visual acuity increased from 0.06 to 0.6 (spectacle-corrected visual acuity was 1.0) and her EDSS score decreased from 4 to 3 points. The other five patients had no clinical improvement and no EDSS scores changes after PE. Adverse events included rashes (P1, P3), acute non-occlusive thrombosis of the internal jugular vein (P1), and thrombocytopenia (P2). In conclusion, the timing of PE initiation as a rescue therapy for severe and corticosteroid-unresponsive pediatric AQP4-IgG positive NMOSD may be crucial to treatment efficacy, and early initiation of PE may be associated with a better outcome. Furthermore, PE has the potential risk for clinically significant adverse effects that should be considered before initiating the therapy and should be weighed against potential benefits. Frontiers Media S.A. 2023-01-05 /pmc/articles/PMC9849793/ /pubmed/36685590 http://dx.doi.org/10.3389/fimmu.2022.1113406 Text en Copyright © 2023 Li, Wan, Liu, Wang, Shi, Zhou, Xu, Wei and Yang 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, Zhichao
Wan, Lin
Liu, Xinting
Wang, Jing
Shi, Xiuyu
Zhou, Huanfen
Xu, Quangang
Wei, Shihui
Yang, Guang
Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder
title Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder
title_full Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder
title_fullStr Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder
title_full_unstemmed Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder
title_short Safety and efficacy of plasma exchange treatment in children with AQP4-IgG positive neuromyelitis optica spectrum disorder
title_sort safety and efficacy of plasma exchange treatment in children with aqp4-igg positive neuromyelitis optica spectrum disorder
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9849793/
https://www.ncbi.nlm.nih.gov/pubmed/36685590
http://dx.doi.org/10.3389/fimmu.2022.1113406
work_keys_str_mv AT lizhichao safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT wanlin safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT liuxinting safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT wangjing safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT shixiuyu safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT zhouhuanfen safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT xuquangang safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT weishihui safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder
AT yangguang safetyandefficacyofplasmaexchangetreatmentinchildrenwithaqp4iggpositiveneuromyelitisopticaspectrumdisorder