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Open Access Highly Accessed Research article

Therapeutic options for chronic inflammatory demyelinating polyradiculoneuropathy: a systematic review

Richard J Bright123*, Jenny Wilkinson2 and Brendon J Coventry3

Author Affiliations

1 Faculty of Health Sciences, School of Dentistry, University of Adelaide, Adelaide, Australia

2 School of Biomedical Sciences, Charles Sturt University, Wagga Wagga, Australia

3 Faculty of Health Sciences, Immunotherapy Research Laboratory, Royal Adelaide Hospital, Adelaide, Australia

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BMC Neurology 2014, 14:26  doi:10.1186/1471-2377-14-26

Published: 7 February 2014

Abstract

Background

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. The aim of this study was to prospectively review the literature to determine the effectiveness of therapies for chronic inflammatory demyelinating polyradiculoneuropathy.

Methods

Articles 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.

Results

Nine 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.

Conclusion

IVIg 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.

Keywords:
Chronic inflammatory demyelinating polyneuropathy; Peripheral neuropathy; Anti-myelin associated glycoprotein; Autoimmune diseases; Treatment; Plasmapheresis; IVIg; Corticosteroids