title: Defective Fas-mediated T-cell apoptosis predicts acute onset CIDP.
abstract: Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) are immune-mediated neuropathies. GBS is characterized by acute onset and subsequent remission of symptoms, whereas CIDP displays slow progression over at least 2 months. However, a small proportion of CIDP patients display acute onset CIDP (a-CIDP) resembling that of GBS. The Fas receptor is involved in shutting off the immune response and its defective function predisposes to auto-immune diseases. In CIDP patients, Fas function is lower than in GBS patients and healthy controls. This study is aimed at assessing whether evaluation of T-cell Fas function helps in early discrimination between GBS and a-CIDP. Fas function was evaluated in patients with acute onset polyneuropathy: 55 retrospective patients analyzed after development of GBS or a-CIDP before year 2005; 50 prospective patients analyzed at onset after year 2005 and followed up for development of GBS or a-CIDP. In both groups, a-CIDP patients displayed defective Fas function, whereas GBS patients displayed normal function. These findings suggest that the evaluation of Fas function in acute onset polyneuropathy helps in early prediction of long-term outcome.
authors.affiliation: Interdisciplinary Research Centre of Autoimmune Diseases (IRCAD), A. Avogadro University of Eastern Piedmont, Novara, Italy. comi@med.unipmn.it
authors.forename: Cristoforo
authors.initials: C
authors.lastname: Comi
authors.forename: Maurizio
authors.initials: M
authors.lastname: Osio
authors.forename: Massimo
authors.initials: M
authors.lastname: Ferretti
authors.forename: Riccardo
authors.initials: R
authors.lastname: Mesturini
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Cappellano
authors.forename: Annalisa
authors.initials: A
authors.lastname: Chiocchetti
authors.forename: Miryam
authors.initials: M
authors.lastname: Carecchio
authors.forename: Caterina
authors.initials: C
authors.lastname: Nascimbene
authors.forename: Claudia
authors.initials: C
authors.lastname: Varrasi
authors.forename: Roberto
authors.initials: R
authors.lastname: Cantello
authors.forename: Claudio
authors.initials: C
authors.lastname: Mariani
authors.forename: Francesco
authors.initials: F
authors.lastname: Monaco
authors.forename: Umberto
authors.initials: U
authors.lastname: Dianzani
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017209:Apoptosis / Q000502:physiology*; D003937:Diagnosis, Differential; D042241:Early Diagnosis; D005260:Female; D005500:Follow-Up Studies; D020275:Guillain-Barre Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000175:diagnosis* / Q000503:physiopathology; D011446:Prospective Studies; D012189:Retrospective Studies; D013601:T-Lymphocytes / Q000502:physiology*; D055815:Young Adult; D019014:fas Receptor / Q000378:metabolism*
chemical_list: D019014:fas Receptor