Complement Inhibition in Myasthenia Gravis and Neuromyelitis Optica Spectrum Disorder

Can J Neurol Sci. 2023 Mar;50(2):165-173. doi: 10.1017/cjn.2021.508. Epub 2021 Dec 13.

Abstract

The complement system is a tightly controlled signaling network that plays a role in innate immune surveillance. However, abnormal signaling through this pathway contributes to tissue damage in several inflammatory, autoimmune, and degenerative diseases. Myasthenia gravis (MG) and neuromyelitis optica spectrum disorders (NMOSD) have complement dysfunction at the core of pathogenesis, providing a strong rationale for therapeutic targeting of complement components. The purpose of this paper is to briefly review the role of complement activation in the pathogenesis of MG and NMOSD, to discuss the rationale and evidence for complement inhibition as a method to manage these diseases, and to provide a Canadian perspective on the use of complement inhibition therapy through real-world cases of MG and NMOSD.

Keywords: Complement system; Eculizumab; Myasthenia gravis; Neuromyelitis optica spectrum disorder.

Publication types

  • Review

MeSH terms

  • Canada
  • Humans
  • Immunologic Factors / therapeutic use
  • Myasthenia Gravis*
  • Neuromyelitis Optica*

Substances

  • Immunologic Factors