Chronic Inflammatory Demyelinating Polyradiculoneuropathy

OBJECTIVE: This article describes the cardinal clinical, electrophysiologic, and supportive features of typical CIDP and its variants, how to review and apply CIDP diagnostic criteria, objective measures that can be used in practice to follow patients with CIDP, and current and emerging treatment practices. LATEST DEVELOPMENTS: The diagnostic criteria for CIDP have been simplified to a diagnosis of either CIDP or possible CIDP. Chronic inflammatory sensory polyradiculoneuropathy is no longer considered a variant of CIDP. Autoimmune nodopathies are considered a separate entity with some clinical and electrodiagnostic features similar to CIDP. Treatments include IV immunoglobulin (IVIg), subcutaneous immunoglobulin, corticosteroids, and plasmapheresis. Subcutaneous efgartigimod alfa, a neonatal Fc-receptor antagonist, was approved by the US Food and Drug Administration (FDA) in June 2024 for the treatment of CIDP, the first novel treatment by drug class for this condition. Complement inhibition is currently being explored in clinical trials for the treatment of CIDP. ESSENTIAL POINTS: CIDP is a rare, treatable neuropathy that is frequently misdiagnosed. As novel therapeutics are studied and approved for clinical use, special attention to the diagnostic criteria and the use of objective treatment outcome measures are essential to preventing misdiagnosis and ensuring that patients receive appropriate therapies.