Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an autoimmune peripheral neuropathy primarily characterized by macrophage-mediated demyelination. Studies have identified that some patients possess autoantibodies against contactin-1 (CNTN1), neurofascin-155 (NF155), contactin-associated protein 1(Caspr1), and neurofascin-186/140 (NF186/140). Based on the unique pathogenesis and pathological features, the 2021 European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) guidelines have categorized these patients separately, defining them as “Autoimmune Nodopathy (AN)”. The standard first-line treatments for CIDP include corticosteroids, intravenous immunoglobulin (IVIG), and plasma exchange. If these treatments are ineffective or poorly tolerated, it can be replaced with immunosuppressants or used in combination. Emerging therapeutic strategies are also being explored, among which subcutaneous injection of efgartigimod, a recently approved drug, is gradually accumulating clinical application value. The treatment strategy for AN differs from that of CIDP: Rituximab is currently regarded as the preferred option for treating AN, with corticosteroids being effective for some patients. Plasma exchange can be utilized for severe cases, while IVIG is largely ineffective for most patients with AN. Due to the low incidence of AN and the limited clinical evidence available, its treatment strategies still require large-scale clinical trials for validation. This article systematically reviews the treatment advancements for CIDP and focuses on the unique treatment strategies for AN.
Pang et al. (2026) studied this question.