Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

J Neurol Neurosurg Psychiatry. 2019 Sep;90(9):981-987. doi: 10.1136/jnnp-2019-320314. Epub 2019 Apr 16.


Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several 'atypical' subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and 'distal acquired demyelinating symmetric neuropathy', possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

Keywords: CIDP; epidemiology; immune neuropathy; neuroimaging; peripheral nerve.

Publication types

  • Review

MeSH terms

  • Autoantibodies / immunology
  • Drosophila Proteins
  • Humans
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / etiology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy
  • T-Box Domain Proteins


  • Autoantibodies
  • Drosophila Proteins
  • H15 protein, Drosophila
  • T-Box Domain Proteins