Chronic inflammatory demyelinating polyradiculoneuropathy-Diagnostic pitfalls and treatment approach

Muscle Nerve. 2021 Feb;63(2):157-169. doi: 10.1002/mus.27046. Epub 2020 Sep 11.


Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by progressive weakness and sensory loss, often affecting patients' ability to walk and perform activities of daily living independently. With the lack of a diagnostic biomarker, the diagnosis relies on clinical suspicion, clinical findings, and the demonstration of demyelinating changes on electrodiagnostic (EDx) testing and nerve pathology. As a result, patients can often be misdiagnosed with CIDP and unnecessarily treated with immunotherapy. Interpreting the EDx testing and cerebrospinal fluid findings in light of the clinical phenotype, recognizing atypical forms of CIDP, and screening for CIDP mimickers are the mainstays of the approach to patients suspected of having CIDP, and are detailed in this review. We also review the currently available treatment options, including intravenous immunoglobulin (IVIg), corticosteroids (CCS), and plasma exchange (PE), and discuss how to approach treatment-refractory cases. Finally, we emphasize the need to adopt objective outcome measures to monitor treatment response.

Keywords: CIDP, diagnosis, IVIg, plasma exchange, steroids, treatment.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Amyloid Neuropathies / diagnosis
  • Cerebrospinal Fluid / chemistry
  • Charcot-Marie-Tooth Disease / diagnosis
  • Diagnosis, Differential
  • Diagnostic Errors
  • Disease Progression
  • Electrodiagnosis*
  • Humans
  • Immunoglobulin G / therapeutic use
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Infusions, Subcutaneous
  • Medical Overuse
  • Neural Conduction*
  • Outcome Assessment, Health Care
  • POEMS Syndrome / diagnosis
  • Paraneoplastic Polyneuropathy / diagnosis
  • Paraproteinemias / complications
  • Paraproteinemias / diagnosis
  • Peripheral Nerves / pathology
  • Peripheral Nerves / physiopathology*
  • Plasma Exchange / methods
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / diagnosis*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / pathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy


  • Adrenal Cortex Hormones
  • Immunoglobulin G
  • Immunoglobulins, Intravenous
  • Immunologic Factors