Unitat Neuromuscular Servei Neurología, Hospital Santa Creu i Sant Pau, Universitat Autònoma Barcelona, CIBERER, Barcelona, Spain.
J Peripher Nerv Syst. 2017 Dec;22(4):418-424. doi: 10.1111/jns.12237. Epub 2017 Nov 15.
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder of the peripheral nerves with clinical and immunological heterogeneity. Both cellular and humoral immune mechanisms against peripheral nerve antigens are considered to contribute to the pathogenesis of the disorder. Currently, the diagnosis of CIDP is based on clinical, laboratory and electrophysiological criteria. The field of CIDP recently underwent a major change with the identification of autoantibodies directed against paranodal (CNTN1, CASPR1 and NF155) and nodal (NF186/140) proteins. Over the last 5 years, correlations have been found between these autoantibodies and CIDP clinical subtypes including the likelihood of response to specific immunotherapies. Additionally, during this time a series of experimental studies have unraveled the underlying immunopathogenesis for CNTN1 and NF155 antibody associated CIDP. Although paranodal and nodal autoantibodies are only found in a small subset of patients with CIDP, the detection of these immune biomarkers should be incorporated in the evaluation of patients, considering the implications of their presence on prognosis, follow-up, and treatment decisions.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种自身免疫性周围神经疾病，具有临床和免疫学异质性。针对周围神经抗原的细胞和体液免疫机制被认为有助于该疾病的发病机制。目前，CIDP 的诊断基于临床、实验室和电生理学标准。CIDP 领域最近发生了重大变化，鉴定了针对神经节段（CNTN1、CASPR1 和 NF155）和结段（NF186/140）蛋白的自身抗体。在过去的 5 年中，这些自身抗体与 CIDP 临床亚型之间发现了相关性，包括对特定免疫疗法反应的可能性。此外，在此期间，一系列实验研究揭示了与 CNTN1 和 NF155 抗体相关的 CIDP 的潜在免疫发病机制。尽管神经节段和结段自身抗体仅在一小部分 CIDP 患者中发现，但考虑到其存在对预后、随访和治疗决策的影响，这些免疫生物标志物的检测应纳入患者评估中。
