Collet R, Caballero-Ávila M, Querol L
Department of Neurology, Hospital Santa Creu i Sant Pau, Barcelona, Spain.
Department of Neurology, Hospital Santa Creu i Sant Pau, Barcelona, Spain; Neuromuscular Diseases Unit, Department of Neurology, Hospital de la Santa Creu i Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
Rev Neurol (Paris). 2023 Oct;179(8):831-843. doi: 10.1016/j.neurol.2023.02.064. Epub 2023 Mar 10.
Autoimmune neuropathies are a heterogeneous group of rare and disabling diseases in which the immune system targets peripheral nervous system antigens and that respond to immune therapies. This review focuses on Guillain-Barré syndrome, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, polyneuropathy associated with IgM monoclonal gammopathy, and autoimmune nodopathies. Autoantibodies targeting gangliosides, proteins in the node of Ranvier, and myelin-associated glycoprotein have been described in these disorders, defining subgroups of patients with similar clinical features and response to therapy. This topical review describes the role of these autoantibodies in the pathogenesis of autoimmune neuropathies and their clinical and therapeutic importance.
自身免疫性神经病是一组异质性的罕见且致残性疾病,免疫系统针对外周神经系统抗原,且对免疫疗法有反应。本综述聚焦于吉兰-巴雷综合征、慢性炎症性脱髓鞘性多发性神经病、多灶性运动神经病、与IgM单克隆丙种球蛋白病相关的多发性神经病以及自身免疫性结节病。在这些疾病中已描述了针对神经节苷脂、郎飞结中的蛋白质以及髓鞘相关糖蛋白的自身抗体,这些自身抗体定义了具有相似临床特征和治疗反应的患者亚组。本专题综述描述了这些自身抗体在自身免疫性神经病发病机制中的作用及其临床和治疗重要性。