Rituximab in juvenile myasthenia gravis-an international cohort study and literature review.
Details
Serval ID
serval:BIB_6A9CBC1C937C
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Rituximab in juvenile myasthenia gravis-an international cohort study and literature review.
Journal
European journal of paediatric neurology
ISSN
1532-2130 (Electronic)
ISSN-L
1090-3798
Publication state
Published
Issued date
09/2022
Peer-reviewed
Oui
Volume
40
Pages
5-10
Language
english
Notes
Publication types: Journal Article ; Review
Publication Status: ppublish
Publication Status: ppublish
Abstract
Juvenile myasthenia gravis (JMG) is a rare, antibody-mediated disorder of the neuromuscular junction. Treatment strategies in JMG are largely informed by adult MG treatments as the pathophysiology is similar. Rituximab is increasingly considered as a treatment option in refractory JMG but has not yet been systematically investigated in this patient group We conducted a retrospective study from five international centres with expertise in paediatric myasthenia. 10 JMG patients treated with rituximab were identified. Following rituximab treatment all patients had a reduction in JMG-related hospital admissions. At 24 month follow up, 6 patients (60%) had achieved complete stable remission or pharmacological remission and 7 patients were able to reduce immunomodulatory treatment(s). The main side-effect was infusion-related reactions (30%) which resolved in all patients with symptomatic treatment. We compared our cohort to previously reported JMG cases treated with rituximab and noted similar response rates but a slightly higher side-effect profile. Rituximab is a safe and effective treatment option in moderate to severe JMG and most patients have an improvement in MG symptoms post treatment.
Keywords
Adult, Child, Cohort Studies, Humans, Myasthenia Gravis/drug therapy, Retrospective Studies, Rituximab/adverse effects, Treatment Outcome, Juvenile myasthenia gravis, Myasthenia gravis, Paediatric, Rituximab
Pubmed
Web of science
Create date
26/07/2022 13:55
Last modification date
10/10/2023 7:00