Pulse oral corticosteroids in pediatric chronic inflammatory demyelinating polyneuropathy

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Amanda B Rogers1, Craig M Zaidman1, Anne M Connolly2

  • 1Department of Neurology, Washington University School of Medicine, St. Louis, Missouri, USA.

Muscle & Nerve
|September 7, 2020
PubMed

Insights

High-dose weekly corticosteroids, or pulse oral corticosteroids, offer a safe and effective long-term treatment for childhood chronic inflammatory demyelinating polyneuropathy (CIDP). This approach showed comparable outcomes to other therapies in pediatric patients.

Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Background:

  • Childhood onset chronic inflammatory demyelinating polyneuropathy (CIDP) typically necessitates prolonged immunomodulatory treatment.
  • Long-term treatment strategies for pediatric CIDP are not extensively documented.

Purpose of the Study:

  • To review the treatment and outcomes of pediatric CIDP patients.
  • To evaluate the efficacy and safety of high-dose weekly corticosteroids (pulse oral corticosteroids) as a maintenance therapy for pediatric CIDP.

Main Methods:

  • Retrospective review of medical records for pediatric patients diagnosed with CIDP between 2000 and 2018.
  • Analysis included demographics, disease course, treatment regimens, and at least 12 months of follow-up data.
  • Focus on patients treated with pulse oral corticosteroids.

Main Results:

  • Twenty-five pediatric patients with CIDP were analyzed.
  • Pulse oral corticosteroid monotherapy was the primary maintenance treatment in 56% of cases.
  • Median follow-up was 4 years; side effects were infrequent.
  • Outcomes (normal exam or off treatment) were similar across different maintenance therapies.

Conclusions:

  • Pulse oral corticosteroid therapy is a safe and effective long-term treatment option for children with CIDP.
  • This treatment approach yields comparable long-term outcomes to other therapies.
  • Further research can explore its role in managing pediatric CIDP.

Keywords:
CIDPcorticosteroidsimmunomodulatory therapyinflammatory neuropathypediatric CIDP

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