Chronic inflammatory demyelinating polyneuropathy: Plasmapheresis or cyclosporine can be good treatment options in

WooJoong Kim1, Young Kyu Shim1, Sun Ah Choi2

  • 1Department of Pediatrics, Seoul National University College of Medicine, Pediatric Clinical Neuroscience Center, Seoul National University Children's Hospital, Seoul, Republic of Korea.

Insights

For refractory childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), plasmapheresis is effective for monophasic cases, while cyclosporine shows promise for polyphasic cases. Early treatment selection may improve outcomes.

Area of Science:

  • Neurology
  • Pediatrics
  • Immunology

Background:

  • Childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare, with unclear optimal treatment, especially for refractory cases.
  • Treatment strategies for pediatric CIDP require further investigation due to its rarity and variable course.

Purpose of the Study:

  • To analyze clinical features and treatment outcomes in childhood CIDP.
  • To identify effective treatments for refractory pediatric CIDP, differentiating between monophasic and polyphasic courses.

Main Methods:

  • Retrospective analysis of 14 childhood CIDP cases with over 12 months follow-up.
  • Comparison of treatment responses (IVIG, plasmapheresis, immunosuppressants) based on disease course (monophasic vs. polyphasic) and refractoriness.

Main Results:

  • Ten of 14 cases were refractory to first-line treatments.
  • Plasmapheresis showed better response than IVIG in monophasic CIDP, particularly in refractory cases.
  • Cyclosporine was effective in controlling disease in treatment-refractory polyphasic CIDP patients.

Conclusions:

  • Early plasmapheresis may benefit monophasic childhood CIDP.
  • Cyclosporine can be an effective option for refractory polyphasic childhood CIDP.
  • Tailoring treatment based on disease course is crucial for refractory pediatric CIDP.

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