Role of complement in myasthenia gravis.
Abstract
Abstract
Myasthenia gravis is a prototypic neuroimmune disorder with autoantibodies targeting the acetylcholine receptor complex at the neuromuscular junction. Patients present with mainly ocular muscle weakness and tend to have a generalized muscle weakness later in the clinical course. The weakness can be severe and fatal when bulbar muscles are heavily involved. Acetylcholine receptor antibodies are present in the majority of patients and are of IgG1 and IgG3 subtypes which can activate the complement system. The complement involvement plays a major role in the neuromuscular junction damage and the supporting evidence in the literature is described in this article. Complement therapies were initially studied and approved for paroxysmal nocturnal hemoglobinuria and in the past decade, those have also been studied in myasthenia gravis. The currently available randomized control trial and real-world data on the efficacy and safety of the approved and investigational complement therapies are summarized in this review.
Keywords: AChR antibody; C5; complement; eculizumab; meningococcal; myasthenia gravis; ravulizumab; zilucoplan.
Author
Citations
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Date
2023-10-05
Type
Article
Subject
Neurology, Pharmacology
Collections
Citation
San PP, Jacob S. Role of complement in myasthenia gravis. Front Neurol. 2023 Oct 5;14:1277596. doi: 10.3389/fneur.2023.1277596. PMID: 37869140; PMCID: PMC10585143.
Journal / Source Title
Frontiers in Neurology
DOI
10.3389/fneur.2023.1277596
PMID
37869140
Publisher
Frontiers Media