title: Autoantibody profile of primary sclerosing cholangitis.
abstract: Primary sclerosing cholangitis (PSC) is a chronic progressive liver disease of unknown etiology. It has been suggested that genetic and immunological factors are important in its pathogenesis. The present study examined the prevalence of 23 different autoantibodies in 25 PSC sera, by ELISA, in order to better define the autoimmune profile of PSC. The results indicate that 88% of PSC patients produced at least 1 autoantibody, and 36% had reactivity to multiple autoantibodies. Moreover, 35% of the PSC patients produced anti-endothelial-cell antibodies (AECA) and 75% of the sera contained perinuclear antineutrophil cytoplasmic antibodies (pANCA), detected by indirect immunofluorescence. The prominent ANCA autoantibody was anti-cathepsin-G, demonstrated in 35% of the patients. The multiplicity of the autoantibody profile, revealed in the present study, points to the autoimmune characteristics of PSC. In addition, the association of ANCA and of AECA in PSC may suggest a pathogenic role for these antibodies in PSC.
authors.affiliation: Department of Medicine, Sheba Medical Center, Tel Hashomer, Israel.
authors.forename: H
authors.initials: H
authors.lastname: Gur
authors.forename: G
authors.initials: G
authors.lastname: Shen
authors.forename: M
authors.initials: M
authors.lastname: Sutjita
authors.forename: J
authors.initials: J
authors.lastname: Terrberry
authors.forename: I
authors.initials: I
authors.lastname: Alosachie
authors.forename: N
authors.initials: N
authors.lastname: Barka
authors.forename: H C
authors.initials: HC
authors.lastname: Lin
authors.forename: J B
authors.initials: JB
authors.lastname: Peter
authors.forename: P L
authors.initials: PL
authors.lastname: Meroni
authors.forename: M
authors.initials: M
authors.lastname: Kaplan
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D056649:Cathepsin G; D002403:Cathepsins / Q000276:immunology; D015209:Cholangitis, Sclerosing / Q000276:immunology*; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D012697:Serine Endopeptidases
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D015415:Biomarkers; C087589:anti-endothelial cell antibody; D002403:Cathepsins; D012697:Serine Endopeptidases; C534094:CTSG protein, human; D056649:Cathepsin G