Therapeutic options for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review

Richard J Bright,Brendon J Coventry,Jenny Wilkinson

doi:10.1186/1471-2377-14-26

Richard J Bright, Brendon J Coventry + Show 1 more

Open Access

https://doi.org/10.1186/1471-2377-14-26

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Abstract

BackgroundChronic inflammatory demyelinating polyradiculoneuropathy is a rare acquired immune-mediated progressive or relapsing disorder causing peripheral neuropathic disease of duration more than two months. Many individuals with chronic inflammatory demyelinating polyradiculoneuropathy fail to make a long-term recovery with current treatment regimes. The aim of this study was to prospectively review the literature to determine the effectiveness of therapies for chronic inflammatory demyelinating polyradiculoneuropathy.MethodsArticles published from January 1990 to December 2012 were searched for studies to treat adults with chronic inflammatory demyelinating polyradiculoneuropathy. Peer-reviewed full-text articles published in English were included.ResultsNine placebo-controlled double-blinded randomised trials were reviewed to treat subjects with chronic inflammatory demyelinating polyradiculoneuropathy exhibiting various degrees of effectiveness. The most effect treatments were; three randomised controlled trials using intravenous immunoglobulin, a study comparing pulsed dexamethasone and short term prednisolone and rituximab all showed promising results and were well tolerated.ConclusionIVIg and corticosteroids remain first line treatments for CIDP. Therapies using monoclonal antibodies, such as Rituximab and Natalizumab offer the most promise for treatment of Chronic inflammatory demyelinating polyradiculoneuropathy however they also need further research, as does the use of stem cell therapy for treating Chronic inflammatory demyelinating polyradiculoneuropathy. Large randomised controlled trials and better patient selection are required to address responsiveness of CIDP patients to conventional treatments to elucidate mechanisms of action and future directions for therapeutic improvement.

Highlights

Chronic inflammatory demyelinating polyradiculoneuropathy is a rare acquired immune-mediated progressive or relapsing disorder causing peripheral neuropathic disease of duration more than two months
As numerous carbohydrate epitopes are shared by melanoma cells and myelin molecular mimicry may be a key factor in the initiation of the condition
All current and emerging treatments for Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were included in the study and papers were excluded if the diagnosis of CIDP was considered secondary to an underlying disorder

Summary

Introduction

Chronic inflammatory demyelinating polyradiculoneuropathy is a rare acquired immune-mediated progressive or relapsing disorder causing peripheral neuropathic disease of duration more than two months. Many individuals with chronic inflammatory demyelinating polyradiculoneuropathy fail to make a long-term recovery with current treatment regimes. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired peripheral neuropathy, with both T and B cell involvement [1]. It is the most common peripheral autoimmune demyelinating neuropathy with a prevalence of 1.2 to 7.7 per 100,000 worldwide, with a slight male predominance [2]. The disease involves progressive loss of immunologic tolerance to peripheral nerve strength and sensation, which may explain the variability in clinical presentation [4,5]. It has been suggested that viral and bacterial components have antigenic similarities to the body’s own proteins leading to an auto-immune reaction, or alterations in T cell function [8,9]

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