title: Pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy.
abstract: The acute lesions of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consist of endoneurial foci of chemokine and chemokine receptor expression and T cell and macrophage activation. The myelin protein antigens, P2, P0, and PMP22, each induce experimental autoimmune neuritis in rodent models and might be autoantigens in CIDP. The strongest evidence incriminates P0, to which antibodies have been found in 20% of cases. Failure of regulatory T-cell mechanism is thought to underlie persistent or recurrent disease, differentiating CIDP from the acute inflammatory demyelinating polyradiculoneuropathy form of Guillain-Barré syndrome. Corticosteroids, intravenous immunoglobulin and plasma exchange each provide short term benefit but the possible long-term benefits of immunosuppressive drugs have yet to be confirmed in randomised, controlled trials.
authors.affiliation: Department of Clinical Neuroscience, King's College London, Guy's Hospital, London, UK. richard.a.hughes@kcl.ac.uk
authors.forename: Richard A C
authors.initials: RA
authors.lastname: Hughes
authors.forename: David
authors.initials: D
authors.lastname: Allen
authors.forename: Anna
authors.initials: A
authors.lastname: Makowska
authors.forename: Norman A
authors.initials: NA
authors.lastname: Gregson
mesh_terms: D000818:Animals; D000906:Antibodies / Q000276:immunology; D006801:Humans; D009444:Neuritis, Autoimmune, Experimental / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D000906:Antibodies