免疫介导性周围神经病的病理生理学机制及诊疗进展

Immune-mediated neuropathies:pathophysiology and management

免疫介导性周围神经病(IMN)是由于免疫系统的调节紊乱导致周围神经损伤的一组异质性疾病,主要包括吉兰-巴雷综合征、慢性炎性脱髓鞘性多发性神经根神经病、多灶性运动神经病等。该组疾病通过适当的免疫治疗大多能获得临床改善,但部分患者治疗效果仍不尽如人意。因此,研究疾病发生发展的病理生理学可揭示疾病的本质,为疾病的预防、诊断和治疗提供理论基础。本文详细综述各种IMN的病理生理学机制,以免疫学机制为重点,并扼要介绍各种IMN的诊治进展。

Immune-mediated neuropathies(IMN)are a heterogenous group of disorders affecting the peripheral nervous system,due to dysregulation of the immune system.It mainly includes Guillain-Barre syndrome,chronic inflammatory demyelinating polyradiculoneuropathy,multifocal motor neuropathy and so on.Most of these diseases can be clinically improved by appropriate immunotherapy,but some patients still have unsatisfactory results.Therefore,studying the pathophysiology of the occurrence and development of diseases can reveal the nature of diseases and provide a theoretical basis for the prevention,diagnosis and treatment of diseases.In this paper,the pathophysiological mechanism of various IMNs is described in detail,with emphasis on immunological mechanism,and the progress of diagnosis and treatment of various IMNs is briefly introduced.