title: Challenges in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy.
abstract: Chronic idiopathic demyelinating polyradiculoneuropathy (CIDP) is a rare disease, the most frequent one within the spectrum of the so-called "chronic immune-mediated neuropathies". Challenges in the treatment of CIDP firstly concern its diagnosis, which may be difficult, mainly for the atypical forms. Secondly, challenges encompass the choice of the first-line treatment, such as corticosteroids, intravenous immunoglobulins (IVIg), and plasma exchanges (PE) that have been proven as efficacious by several randomized controlled trials (RCT). Recent reports have focused on both different regimens of corticosteroids, and the occurrence of relapses following treatment with either corticosteroids or IVIg. These data may be helpful for the choice of the first-line treatment and may result in changing the guidelines for treatment of CIDP in clinical practice. The third and more difficult challenge is to manage long-term treatment for CIDP, since no immunomodulatory treatment has to date been proven as efficacious in this situation. Lastly, challenges in the treatment concern the choice of the best outcome measure for CIDP in RCT and clinical practice. The aim of this article is to overview the results of the more recently reported published trials for CIDP, and to give some insights for the current and future management of CIDP.
authors.affiliation: Centre national de référence maladies neuromusculaires rares, CHU Pitié-Salpêtrière, bâtiment Babinski, 47, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
authors.forename: R
authors.initials: R
authors.lastname: Guimarães-Costa
authors.affiliation: Centre national de référence maladies neuromusculaires rares, CHU Pitié-Salpêtrière, bâtiment Babinski, 47, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
authors.forename: R
authors.initials: R
authors.lastname: Iancu Ferfoglia
authors.affiliation: Fédération de neurophysiologie clinique, institut hospitalo-universitaire de neurosciences, groupe hospitalier Pitié-Salpêtrière, université Pierre-et-Marie-Curie (Paris VI), 47, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
authors.forename: K
authors.initials: K
authors.lastname: Viala
authors.affiliation: Centre national de référence maladies neuromusculaires rares, CHU Pitié-Salpêtrière, bâtiment Babinski, 47, boulevard de l'Hôpital, 75651 Paris cedex 13, France. Electronic address: jean-marc.leger@psl.aphp.fr.
authors.forename: J-M
authors.initials: JM
authors.lastname: Léger
mesh_terms: D002986:Clinical Trials as Topic; D006801:Humans; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis / Q000628:therapy*
keywords: Chronic dysimmune neuropathies; Chronic inflammatory demyelinating polyradiculoneuropathy; Immunoglobulines polyvalentes intraveineuses à fortes doses; Immunomodulatory treatments; Intravenous immunoglobulins; Plasma exchanges; Polyneuropathie inflammatoire démyélinisante chronique; Polyneuropathies dysimmunitaires chroniques; Traitements immunomodulateurs; Échanges plasmatiques