慢性免疫介导周围神经病的免疫治疗进展

The Progress of Immunotherapy for Chronic Immune-Mediated Peripheral Neuropathy

摘要: 慢性免疫介导周围神经病是一组由免疫系统调节功能紊乱导致的、以慢性进行性或复发性病程为特征的周围神经疾病，主要包括慢性炎性脱髓鞘性多发性神经根神经病、多灶性运动神经病、抗髓鞘相关糖蛋白抗体相关神经病以及自身免疫性郎飞结病。传统的一线治疗(糖皮质激素、静脉注射免疫球蛋白和血浆置换)虽能使多数患者获益，但仍有约20%~30%的患者疗效不佳、易复发或难以耐受长期治疗。近年来，随着对疾病免疫病理机制认识的深化，靶向B细胞、新生儿Fc受体、补体系统及细胞内信号通路的治疗策略取得了突破性进展，为难治性患者提供了新的选择。本文旨在系统综述慢性免疫介导周围神经病的新型免疫治疗进展，探讨其作用机制、临床证据及未来发展方向，以期为临床实践提供参考。

Abstract: Chronic immune-mediated peripheral neuropathies are a group of peripheral nerve disorders characterized by chronic progressive or relapsing courses, resulting from dysregulation of the immune system. They mainly include chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, anti-myelin-associated glycoprotein antibody-related neuropathy, and autoimmune nodopathies. Although conventional first-line therapies (glucocorticoids, intravenous immunoglobulin, and plasma exchange) benefit most patients, approximately 20%~30% of patients still show poor response, experience relapses, or have difficulty tolerating long-term treatment. In recent years, with a deeper understanding of the immunopathological mechanisms underlying these diseases, breakthrough progress has been made in therapeutic strategies targeting B cells, the neonatal Fc receptor, the complement system, and intracellular signaling pathways, offering new options for refractory patients. This article aims to systematically review the advances in novel immunotherapies for chronic immune-mediated peripheral neuropathies, discuss their mechanisms of action, clinical evidence, and future directions, with the goal of providing a reference for clinical practice.