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Determining Immune System Suppression versus CNS Protection for Pharmacological Interventions in Autoimmune Demyelination
Published on: September 12, 2016
Different types of chronic inflammatory demyelinating polyneuropathy have a different clinical course and response to
Stefano Jann1, Sandro Beretta, Manuela A Bramerio
1Department of Neurology, Niguarda Hospital, Piazza Ospedale Maggiore 3, I-20162 Milan, Italy. stefano.jann@fastwebnet.it
Abstract:
Chronic inflammatory demyelinating polyneuropathy (CIDP) can occur in association with other systemic diseases such as diabetes mellitus (DM) and IgG or IgA monoclonal gammopathy of undetermined significance (MGUS). Whether CIDP that is idiopathic (I-CIDP) or associated with diabetes (CIDP-DM) or MGUS (CIDP-MGUS) differ in clinical presentation, laboratory features, response to treatment, and long-term outcome is unclear, as is the relationship between these coexisting diseases and CIDP. In order to clarify this issue, we began a prospective follow-up study. Thirty-one consecutive patients with untreated CIDP, fulfilling the most restrictive diagnostic criteria, were enrolled over 18 months. Among the patients, 16 were diabetic, 7 had a MGUS, and 8 had an idiopathic CIDP. All patients were treated with IVIg, and the responders were treated again if they relapsed. In all three groups, improvement occurred after treatment. At the end of the follow-up, there was no difference in clinical conditions between groups, but a significant difference existed in the number of relapses and of IVIg administrations. CIDP-DM is a more severe disease, but with a significantly better response to IVIg and fewer relapses, than the other types that we studied.
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