title: A comprehensive evaluation of serum autoantibodies in primary biliary cirrhosis.
abstract: In primary biliary cirrhosis (PBC) serum markers other than anti-mitochondrial antibodies (AMA) are promising in terms of disease severity and comorbidities, as well represented by anti-nuclear antibodies (ANA). The aim of the present study was thus to evaluate the prevalence and clinical significance of a large profile of serum autoantibodies in PBC sera. We utilized 69 sera from European patients with PBC (including 20 AMA-negative) and 297 sera from geographically and sex-matched healthy controls. All sera were tested for the presence of ANA and autoantibodies associated with thrombophilia, vasculitis, and gastrointestinal disease. Autoantibodies other than AMA were detected in 53/69 (76%) PBC sera vs. 105/297 (35%) among controls. The prevalence of ANA (targeting dsDNA, Sm, chromatin, ribosomal-P, RNP, SmRNP, SSA, SSB, and centromere) and thrombophilia-associated autoantibodies (i.e. anti-beta2GPI, phosphatydilserine, prothrombin) was common among patients with PBC. When clinical features were compared, the presence of anti-prothrombin IgM was associated with a worse prognosis as represented by a higher Mayo score. We demonstrate an increased prevalence of ANA and thrombophilia-associated autoantibodies in PBC sera and an association between the latter autoantibodies and PBC stage. The role of thrombophilia-associated antibodies will warrant further studies, based in particular on the incidence of portal hypertension at early stages of PBC.
authors.affiliation: Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer 52621, Israel.
authors.forename: Nancy
authors.initials: N
authors.lastname: Agmon-Levin
authors.forename: Yinon
authors.initials: Y
authors.lastname: Shapira
authors.forename: Carlo
authors.initials: C
authors.lastname: Selmi
authors.forename: Ori
authors.initials: O
authors.lastname: Barzilai
authors.forename: Maya
authors.initials: M
authors.lastname: Ram
authors.forename: Martine
authors.initials: M
authors.lastname: Szyper-Kravitz
authors.forename: Sara
authors.initials: S
authors.lastname: Sella
authors.forename: Bat-Sheva Porat
authors.initials: BS
authors.lastname: Katz
authors.forename: Pierre
authors.initials: P
authors.lastname: Youinou
authors.forename: Yves
authors.initials: Y
authors.lastname: Renaudineau
authors.forename: Bruno
authors.initials: B
authors.lastname: Larida
authors.forename: Pietro
authors.initials: P
authors.lastname: Invernizzi
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
authors.forename: Yehuda
authors.initials: Y
authors.lastname: Shoenfeld
mesh_terms: D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood*; D003430:Cross-Sectional Studies; D018450:Disease Progression; D005260:Female; D006801:Humans; D007075:Immunoglobulin M / Q000097:blood; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000175:diagnosis* / Q000276:immunology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011516:Prothrombin / Q000276:immunology; D018570:Risk Assessment; D019851:Thrombophilia; D014657:Vasculitis
chemical_list: D000974:Antibodies, Antinuclear; D007075:Immunoglobulin M; D011516:Prothrombin