中国医学创新2024，Vol.21Issue(33)：184-188,5.DOI:10.3969/j.issn.1674-4985.2024.33.044
自身免疫性郎飞结病的研究进展
Research Progress on Autoimmune Nodopathies
摘要
Abstract
The node of Ranvier is the portion of a myelinated nerve fiber that is not myelinated between every two Schwann cells in a myelinated nerve fiber.It contains four regions:the junctional,parajunctional,subparajunctional,and interjunctional regions.Autoimmune nodopathy(AN)is a peripheral neuropathy with motor-sensory involvement mediated by antibodies related to neurofascin(NF)140/186 in the junctional region of the node of Ranvier,NF155 located in the myelin sheath in the parajunctional region of the node,contactin-1(CNTN1)and contactin-associated protein-1(Caspr1)located in the axon.AN is similar to CIDP in clinical manifestations,but there are differences in pathologic features and therapeutic responses,thus providing unique diagnostic and therapeutic value.Currently,glucocorticoids,plasma exchange and intravenous immunoglobulin(IVIG)are the mainstays of treatment for patients with AN,but patients with IgG4 subtypes of AN are poorly treated with immunoglobulin,and targeted biologics,such as Eculizumab and Bortezomib,have also shown some efficacy.This review focuses on the clinical manifestations as well as therapeutic advances in AN.关键词
自身免疫性郎飞结病/神经束蛋白140/186/神经束蛋白155/接触蛋白1/接触蛋白相关蛋白1Key words
AN/NF140/186/NF155/CNTN1/Caspr1引用本文复制引用
卢璐,宋艳..自身免疫性郎飞结病的研究进展[J].中国医学创新,2024,21(33):184-188,5.基金项目
山东省中医药科技发展计划项目(2019-0491) （2019-0491）
济宁医学院附属医院医学英才培养计划项目(jyfy-yc-004) （jyfy-yc-004）
