Autoimmune Diseases Volume 2014 (2014), Article ID 201657, 11 pageshttp://dx.doi.org/10.1155/2014/201657
Review Article Treatment of Chronic Inflammatory Demyelinating Polyneuropathy: From Molecular Bases to Practical Considerations
Paolo Ripellino,1 Thomas Fleetwood,1 Roberto Cantello,1 and Cristoforo Comi1,2,3
1 Department of Neurology, A.O.U. Maggiore di Novara, Amedeo Avogadro University, Corso Mazzini 18, 28100 Novara, Italy
2 Department of Translational Medicine, Section of Neurology and Interdisciplinary Research Centre of Autoimmune Diseases (IRCAD), Via Solaroli 17, 28100 Novara, Italy
3 Department of Translational Medicine, Amedeo Avogadro University, Via Solaroli 17, 28100 Novara, Italy
Received 29 September 2013; Accepted 13 November 2013; Published 14 January 2014
Academic Editor: Umberto Dianzani
Copyright © 2014 Paolo Ripellino et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, in which both cellular and humoral immune responses are involved. The disease is clinically heterogeneous with some patients displaying pure motor form and others also showing a variable degree of sensory dysfunction; disease evolution may also differ from patient to patient, since monophasic, progressive, and relapsing forms are reported.
Underlying such clinical variability there is probably a broad spectrum of molecular dysfunctions that are and will be the target of therapeutic strategies. In this review we first explore the biological bases of current treatments and subsequently we focus on the practical management that must also take into account pharmacoeconomic issues.
[snip]CIDP is defined by a slow clinical deterioration that reaches its maximum after more than 8 weeks, differently from GBS, which is an acute and self-limiting disease. That aside, there are many similarities between these two conditions, which may even be variants of the same disease spectrum, with CIDP being the result of prolonged survival of activated T cells
, not undergoing apoptosis due to a defective Fas pathway function [18–20], and GBS characterized by a self-limitation likely related to a preserved function of such apoptotic mechanism. In line with this concept, the finding that corticosteroids are effective in CIDP and not in GBS would be related to the known effect of these drugs in restoring T cell apoptosis.
Currently available treatments for CIDP are corticosteroids, immune globulin, plasma exchange (PE), and chronic immunosuppressive agents [21, 25].
CIDP is a rare but treatable disease. Clinical experience indicates that about 70% of patients will respond to immunomodulation; there are patients responding to steroids, whereas others, especially with pure motor CIDP, will benefit more from IVIg or PE. It is becoming evident that CIDP is not a uniform disease but includes several variants which might have different response profiles