Pediatric CIDP: Clinical Features and Response to Treatment

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Abstract

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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