The chronic inflammatory neuropathies (CINs) are rare, very disabling autoimmune disorders that generally respond well to immune therapies such as intravenous immunoglobulin (IVIg). The most common forms of CIN are chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy, and polyneuropathy associated with monoclonal gammopathy of unknown significance. The field of CIN has undergone a major advance with the identification of IgG4 autoantibodies directed against paranodal proteins in patients with CIDP. Although these autoantibodies are only found in a small subset of patients with CIDP, they can be used to guide therapeutic decision-making, as these patients have a poor response to IVIg. These observations provide proof of concept that identifying the target antigens in tissue-specific antibody-mediated autoimmune diseases is important, not only to understand their underlying pathogenic mechanisms, but also to correctly diagnose and treat affected patients. This state-of-the-art Review focuses on the role of autoantibodies against nodes of Ranvier in CIDP, a clinically relevant emerging field of research. The role of autoantibodies in other immune-mediated neuropathies, including other forms of CIN, primary autoimmune neuropathies, neoplasms, and systemic diseases that resemble CIN, are also discussed.

中文翻译：

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慢性炎性神经病中的自身抗体：诊断和治疗意义

慢性炎性神经病（CIN）很少见，非常致残的自身免疫性疾病，通常对免疫疗法（例如静脉内免疫球蛋白（IVIg））反应良好。CIN的最常见形式是慢性炎症性脱髓鞘性多发性神经根神经病（CIDP），多灶性运动神经病以及与意义不明的单克隆性丙种球蛋白病相关的多发性神经病。CIN领域已取得重大进展，鉴定了CIDP患者中针对旁淋巴结蛋白的IgG4自身抗体。尽管这些自身抗体仅在少数CIDP患者中发现，但由于这些患者对IVIg的反应较差，因此可用于指导治疗决策。这些观察结果提供了概念证明，即在组织特异性抗体介导的自身免疫性疾病中鉴定靶抗原非常重要，不仅要了解其潜在的致病机制，而且对于正确诊断和治疗受影响的患者也很重要。这项最新的综述着重于针对Ranvier淋巴结的自身抗体在CIDP中的作用，CIDP是临床相关的新兴研究领域。还讨论了自身抗体在其他免疫介导的神经病（包括其他形式的CIN，原发性自身免疫神经病，肿瘤和类似于CIN的系统性疾病）中的作用。这项最新的综述着重于针对Ranvier淋巴结的自身抗体在CIDP中的作用，CIDP是临床相关的新兴研究领域。还讨论了自身抗体在其他免疫介导的神经病（包括其他形式的CIN，原发性自身免疫神经病，肿瘤和类似于CIN的系统性疾病）中的作用。这项最新的综述着重于针对Ranvier淋巴结的自身抗体在CIDP中的作用，CIDP是临床相关的新兴研究领域。还讨论了自身抗体在其他免疫介导的神经病（包括其他形式的CIN，原发性自身免疫神经病，肿瘤和类似于CIN的系统性疾病）中的作用。