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重症肌无力和视神经脊髓炎谱系障碍中的补体抑制

Complement Inhibition in Myasthenia Gravis and Neuromyelitis Optica Spectrum Disorder.

作者信息

Dodig Dubravka, Genge Angela, Selchen Daniel, Freedman Mark S

机构信息

Division of Neurology, Department of Medicine, University of Toronto and Toronto Western Hospital, Toronto, Ontario, Canada.

Department of Neurology and Neurosurgery, Montreal Neurological Institute, Montreal, Quebec, Canada.

出版信息

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.

摘要

补体系统是一个受到严格调控的信号网络,在先天性免疫监视中发挥作用。然而,通过该途径的异常信号传导会在多种炎症性、自身免疫性和退行性疾病中导致组织损伤。重症肌无力(MG)和视神经脊髓炎谱系障碍(NMOSD)的发病机制核心是补体功能障碍,这为针对补体成分进行治疗提供了有力的理论依据。本文的目的是简要回顾补体激活在MG和NMOSD发病机制中的作用,讨论抑制补体作为治疗这些疾病方法的理论依据和证据,并通过MG和NMOSD的实际病例提供加拿大在使用补体抑制疗法方面的观点。

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