Department of Neurology, The Second Xiangya Hospital, Central South University, Renmin Road 139#, Changsha, 410011, Hunan, China.
J Neurol. 2021 Aug;268(8):2757-2768. doi: 10.1007/s00415-020-09823-2. Epub 2020 Apr 7.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is considered to be an immune-mediated heterogeneous disease involving cellular and humoral immunity. In recent years, autoantibodies against nodal/paranodal protein neurofascin155 (NF155), neurofascin186 (NF186), contactin-1 (CNTN1), and contactin-associated protein 1 (CASPR1) have been identified in a small subset of patients with CIDP, which disrupt axo-glial interactions at nodes/paranodes. Although CIDP electrodiagnosis was made in patients with anti-nodal/paranodal component autoantibodies, macrophage-induced demyelination, the characteristic of typical CIDP, was not observed. Apart from specific histopathology, the pathogenic mechanisms and clinical manifestations of CIDP with autoantibody are also distinct. We herein compared pathogenesis, histopathology, clinical manifestations, and therapeutic response in CIDP with autoantibody vs. CIDP without autoantibody. CIDP with autoantibodies should be considered as an independent disease entity, not a subtype of CIDP due to many differences. They possibly should be classified as CIDP-like chronic nodo-paranodopathy, which can better characterize these disorders, help diagnose and make the most effective therapeutic decisions.
慢性炎症性脱髓鞘性多发性神经病（CIDP）被认为是一种涉及细胞和体液免疫的免疫介导的异质性疾病。近年来，在一小部分 CIDP 患者中发现了针对神经束蛋白 155（NF155）、神经束蛋白 186（NF186）、接触蛋白 1（CNTN1）和接触蛋白相关蛋白 1（CASPR1）的自身抗体，这些自身抗体破坏了神经节/神经旁区的轴突-胶质相互作用。尽管在具有抗神经节/神经旁成分自身抗体的患者中进行了 CIDP 电诊断，但并未观察到典型 CIDP 的特征性巨噬细胞诱导脱髓鞘。除了特定的组织病理学外，具有自身抗体的 CIDP 的发病机制和临床表现也不同。我们在此比较了具有自身抗体的 CIDP 与无自身抗体的 CIDP 的发病机制、组织病理学、临床表现和治疗反应。由于存在许多差异，具有自身抗体的 CIDP 应被视为一种独立的疾病实体，而不是 CIDP 的一种亚型。它们可能应该被归类为 CIDP 样慢性神经束蛋白病，这可以更好地描述这些疾病，有助于诊断并做出最有效的治疗决策。
