Department of Neurology, Faculty of Medicine and University Hospital of Cologne, Kerpener Straße 62, D-50937, Cologne, Germany.
Curr Pharm Des. 2022;28(11):854-862. doi: 10.2174/1381612828666220325102840.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired immune-mediated polyradiculoneuropathy leading to disability via inflammatory demyelination of peripheral nerves. Various therapeutic approaches with different mechanisms of action are established for the treatment of CIDP. Of those, corticosteroids, intravenous or subcutaneous immunoglobulin, or plasma exchange are established first-line therapies as suggested by the recently revised EAN/PNS guidelines for the management of CIDP. In special cases, immunosuppressants or rituximab may be used. Novel therapeutic approaches currently undergoing clinical studies include molecules or monoclonal antibodies interacting with Fc receptors on immune cells to alleviate immune-mediated neuronal damage. Despite various established therapies and the current development of novel therapeutics, treatment of CIDP remains challenging due to an heterogeneous disease course and the lack of surrogate parameters to predict the risk of clinical deterioration. This review summarizes established therapies for CIDP and provides an outlook on future therapeutic approaches.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种获得性免疫介导的多发性神经根神经病，通过外周神经的炎症性脱髓鞘导致残疾。目前已经确立了各种具有不同作用机制的治疗方法来治疗 CIDP。其中，根据最近修订的 EAN/PNS 管理 CIDP 指南建议，皮质类固醇、静脉或皮下免疫球蛋白或血浆置换是既定的一线治疗方法。在特殊情况下，可能会使用免疫抑制剂或利妥昔单抗。目前正在进行临床研究的新型治疗方法包括与免疫细胞上的 Fc 受体相互作用的分子或单克隆抗体，以减轻免疫介导的神经元损伤。尽管有多种既定的治疗方法和目前新型治疗方法的发展，但由于疾病过程的异质性和缺乏预测临床恶化风险的替代参数，CIDP 的治疗仍然具有挑战性。本文综述了 CIDP 的既定治疗方法，并展望了未来的治疗方法。
