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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.
Abstract:
Childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare condition, and the optimal treatment strategy is not well established, especially in refractory cases. We analyzed the clinical features and treatment outcomes of 14 cases of childhood CIDP with more than 12 months of follow-up. Of the 14 cases, 10 cases were considered refractory to the conventional first-line treatment. In the monophasic group (n = 6), plasmapheresis resulted in a better treatment response than did IVIG. Monophasic refractory cases (n = 4) were especially responsive to plasmapheresis. In the polyphasic group (n = 8), IVIG and plasmapheresis had comparable effects. Among them six polyphasic patients were refractory to the first-line treatment options and received additional immunosuppressants. Four treatment-refractory polyphasic patients received cyclosporine and achieved successful disease control. With regard to the long-term outcomes, six patients showed minimal symptoms and no relapse within 6 months. Our results suggest that early administration of plasmapheresis in a monophasic course and cyclosporine in a polyphasic course may be effective treatment options for refractory childhood CIDP.
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