摘要自身免疫性结旁病（APN）是一类以结旁区重要蛋白复合体为攻击靶点、自身抗体介导的少见独立实体性疾病。致病抗体包括神经束蛋白155抗体、接触蛋白1抗体及接触蛋白相关蛋白1 抗体。尽管APN与慢性炎性脱髓鞘性多发性神经根神经病（CIDP）患者存在诸多共性，但APN患者也具有相对特征性的临床特征、发病机制、病理改变和对静脉注射免疫球蛋白治疗反应，从而有别于经典的CIDP。APN的致病抗体以IgG4亚型为主，其他IgG亚型罕见。及时对患者进行APN抗体的筛查及IgG分型，不但有助于明确诊断，且可对精准治疗的选择和判断预后提供有价值的临床信息。

abstractsAutoimmune paranodopathy (APN) has emerged as an independent rare disease,which is medicated by autoimmune antibodies against the essential complex of paranodal region of Ranvier. The antibodies include anti-neurofascin 155 antibody, anti-contactin-1 antibody and anti-contactin-associated protein 1 antibody. Although there are many similarities between APN and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), patients with APN have relatively unique clinical features, pathogenesis, histopathological results and responses to intravenous immune globulin, distinguishing from typical CIDP. The predominant subclass of IgG among pathogenic antibodies is IgG4, meanwhile, other subclasses have been rarely reported. Early detecting the APN related antibodies and their subclasses not only helps to clarify the diagnosis, but also provides valuable clinical information for the selection of precise treatment and prognosis.