ABSTRACT
Introduction: Chronic inflammatory neuropathies are disorders caused by an immune response to peripheral nerve. They include chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), multifocal motor neuropathy (MMN) and neuropathy associated with anti-MAG IgM monoclonal gammopathy and other less frequent neuropathies. Several immune therapies have been proven to be effective in these neuropathies even if the best therapeutic option is still unsettled.
Areas covered: The authors reviewed the literature to compare the efficacy and safety of currently used immune therapies in these neuropathies. The authors also analyzed the effect of other immune suppressive agents and of biological agents including rituximab, eculizumab, natalizumab, alemtuzumab and fingolimod that were found effective in other autoimmune diseases.
Expert commentary: Despite the reported efficacy of a number of new immune therapies in some patients with immune mediated neuropathies, their efficacy has not been so far confirmed in randomized controlled studies. High-dose intravenous immunoglobulin (IVIg) (and subcutaneous immunoglobulin [SCIg] for maintenance treatment), steroids and plasma exchange remain the only therapy of proven efficacy in CIDP, IVIg in MMN and, with certain limits, rituximab and, occasionally plasma exchange in neuropathy associated with anti-MAG antibodies. New biological agents are also on the horizon but their efficacy needs to be proved in controlled studies.
Declaration of interest
E. Nobile-Orazio reports personal compensation for serving on the Steering Committee or Advisory Board of: Baxter, Italy; CSL Behring, Italy and USA; Kedrion Biopharma, Italy; LFB, France; Novartis, Switzerland; UCB, UK. He has received honoraria for lecturing from Baxter, USA and Italy, CSL Behring, Italy, Kedrion Biopharma, Italy, Grifols, Spain and travel supports for Scientific Meetings from CSL Behring, Italy and Kedrion Biopharma, Italy. All compensations and supports are outside the submitted work. F. Gallia, F. Terenghi and M. Bianco received travel supports for Scientific Meetings from CSL Behring, Italy and Kedrion Biopharma, Italy. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.