Treatment of chronic inflammatory demyelinating polyneuropathy

Muscle Nerve. 2022 Nov;66(5):552-557. doi: 10.1002/mus.27709. Epub 2022 Sep 6.

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic peripheral polyneuropathy that results in disability through immune-mediated nerve injury, but which not uncommonly has residual and irreversible neurological deficits after the active inflammatory component of the disorder has been treated. Management of the condition entails addressing both the abnormal immune activity that drives ongoing or active deficits while also managing residual symptoms through supportive interventions. Immune-based treatments are grounded in several important principles. First, early treatment is guided by evidence-based, proven-effective therapies that sequentially escalate depending on the response. Second, optimization or personalization of first-line treatments is needed to understand the ideal dose for any given patient, and whether long-term treatment is needed at all. Third, although many immunosuppressive agents may be utilized in nonresponding patients or when intravenous immunoglobulin (IVIg)/corticosteroid-sparing intervention is desired, all are unproven and require a delicate balance between risk, cost, and unknown likelihood of benefit that is tailored to each individual patient's unique circumstances. There is no reliable disease activity biomarker that can be used to guide treatment---a reality that makes it very challenging to optimize treatment to individual patient needs. Serial clinical assessments are key to understanding the value of continued immunotherapy or if long-term therapy is needed at all. Regardless of the immunotherapy status of a patient, equally important is addressing residual deficits through supportive interventions, including physical therapy, adaptive equipment, pain management, and emotional support.

Keywords: chronic inflammatory demyelinating polyneuropathy; immunotherapy; misdiagnosis; outcome measure; treatment.

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Immunologic Factors / therapeutic use
  • Immunosuppressive Agents / therapeutic use
  • Polyneuropathies* / drug therapy
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* / drug therapy
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating* / therapy

Substances

  • Immunoglobulins, Intravenous
  • Immunosuppressive Agents
  • Immunologic Factors
  • Adrenal Cortex Hormones