2024 年 41 巻 3 号 p. 343-348
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired neuropathy resulting from an autoimmune response targeting the myelin sheath within the peripheral nervous system. It manifests as slowly progressive or relapsing motor and sensory disturbances in the limbs. The heterogeneous nature of symptom distribution and impairment modalities suggests a broad clinical spectrum, complicating the attribution of a single underlying pathology.
First–line treatments for CIDP include high–dose intravenous immunoglobulins, corticosteroids, and plasmapheresis. Although these therapies vary in efficacy, they are all recognized as effective for typical CIDP presentations. Conversely, autoimmune nodopathy, which the EAN/PNS CIDP guidelines have excluded from CIDP classification, constitutes a distinct condition with a common pathogenesis associated with autoantibodies targeting nodal and paranodal regions. Therapies designed to neutralize these autoantibodies could offer new avenues for those with treatment–resistant CIDP or similar clinical manifestations.
This summary outlines the properties and roles of established treatments and introduces emerging therapies, including FcRn inhibitors, rituximab, agents targeting the classical complement pathway, and advanced B cell depletion strategies such as BTK inhibitors and chimeric autoantibody receptor T (CAAR–T) cell therapy. The novelty of many recent pharmacological developments lies in their hypothesis–driven targeting of as–yet unidentified autoantibodies, promising valuable contributions to our understanding of the disease's mechanisms.
