2025 年 42 巻 4 号 p. 559-562
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune–mediated neuropathy typically showing weakness in proximal as well as distal limb muscles, classified as “typical CIDP” in such cases. The EAN/PNS 2021 guideline provided clinical criteria for typical CIDP, and it is important to note that other advanced polyneuropathies besides CIDP may also meet the criteria. In typical CIDP, it is crucial to distinguish the condition from autoimmune nodopathy, diabetic neuropathy, Guillain–Barré syndrome, and myopathy. All first–line immunotherapies (immunoglobulin, corticosteroid, and plasma exchange) are effective for typical CIDP, and typical CIDP is more responsive to immunotherapies compared to other CIDP variants. Optimal regimens for first–line immunotherapies, management strategies for refractory cases, and the role of neonatal Fc receptor–targeted therapies as a novel treatment approach remain areas requiring further investigation.
