Role of complement in myasthenia gravis

Frontiers in Neurology

Neurology

Pyae Phyo San¹  Saiju Jacob² 

[1] Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, United Kingdom;Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, United Kingdom;Department of Neurology, Queen Elizabeth Hospital Birmingham, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom;

关键词: myasthenia gravis;    AChR antibody;    complement;    C5;    eculizumab;    ravulizumab;    zilucoplan;    meningococcal;   

DOI  :  10.3389/fneur.2023.1277596

 received in 2023-08-14, accepted in 2023-09-18,  发布年份 2023

来源: Frontiers

【 摘 要 】

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.

【 授权许可】

Unknown   
Copyright © 2023 San and Jacob.