Pediatric CIDP: Clinical Features and Response to Treatment
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Published on: May 11, 2022
Yohei Harada1, David N Herrmann, Eric L Logigian
1Department of Neurology, University of Rochester School of Medicine and Dentistry, Rochester, NY.
Insights
High-dose oral prednisone shows promise for treating pediatric chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) when intravenous immunoglobulin (IVIg) is ineffective. This treatment was well-tolerated and improved outcomes in non-responders.
Area of Science:
- Pediatric Neurology
- Immunology
- Clinical Therapeutics
Background:
- Childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- While immunomodulatory treatments are effective, optimal therapeutic strategies remain unclear.
Purpose of the Study:
- To evaluate the efficacy and tolerability of pulse oral corticosteroid therapy as an alternative to intravenous immunoglobulin (IVIg) in pediatric CIDP patients.
- To assess treatment responses in children with CIDP who did not respond to initial IVIg therapy.
Main Methods:
- Retrospective review of pediatric CIDP patients (1999-2015) diagnosed using established criteria.
- Tabulation of clinical details and treatment responses, focusing on IVIg and oral corticosteroid interventions.
Main Results:
- Ten pediatric patients (ages 4-16) with definite or possible CIDP were included.
- Six of ten patients initially responded to IVIg; four did not.
- All four IVIg non-responders and one IVIg responder improved with high-dose, twice-weekly oral prednisone, which was well-tolerated.
Conclusions:
- Pulse oral corticosteroid therapy presents a viable alternative treatment option for pediatric CIDP, particularly for IVIg non-responders.
- Further multicenter research is recommended to compare the efficacy and safety of oral corticosteroids versus IVIg in pediatric CIDP.
Objectives:
Childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) responds favorably to immunomodulatory treatment. However, the optimal sequencing and selection of immunotherapy is uncertain.
Methods:
Using accepted diagnostic criteria, pediatric patients with CIDP seen at our center from 1999 to 2015 were identified retrospectively through medical record review. Clinical details and treatment responses were tabulated.
Results:
Ten patients (age 4-16, 6 women) with definite (N = 8) or possible (N = 2) CIDP met criteria. All were initially treated with IVIg; 6 responded but 4 did not. All 4 IVIG nonresponders improved with twice-weekly high-dose oral prednisone, as did 1 IVIg responder who was also treated with twice-weekly oral prednisone when IVIg was discontinued. Pulse steroids were well tolerated.
Conclusions:
Pulse oral corticosteroid therapy holds promise as an alternative treatment to IVIG in pediatric CIDP. Future multicenter studies are warranted to determine the comparative efficacy and safety of weekly pulse oral corticosteroids versus IVIg in pediatric CIDP.
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