title: Making Sense of Autoantibodies in Cholestatic Liver Diseases.
abstract: Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the most common chronic cholestatic liver diseases (CLD) in adults and are associated with immune mechanisms. PBC is considered a model autoimmune disease, and more than 90% of patients present very specific autoantibodies against mitochondrial antigens. Whether PSC should be considered an autoimmune or merely immune-mediated disease is still under debate. This review addresses the clinical relevance of autoantibodies in CLD and their pathogenic mechanisms and illustrates the technology available for appropriate autoantibody detection.
authors.affiliation: Liver Unit and Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via A. Manzoni 113, Rozzano, Milan 20089, Italy; Department of Electronics, Information and Bioengineering, Politecnico di Milano, via Ponzio 34/5, Milan 20133, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Marzorati
authors.affiliation: Liver Unit and Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via A. Manzoni 113, Rozzano, Milan 20089, Italy; Division of Rheumatology, Allergy, and Clinical Immunology, University of California Davis, GBSF, 451 Health Science Drive, Davis, CA 95616, USA.
authors.forename: Pietro
authors.initials: P
authors.lastname: Invernizzi
authors.affiliation: Liver Unit and Center for Autoimmune Liver Diseases, Humanitas Clinical and Research Center, Via A. Manzoni 113, Rozzano, Milan 20089, Italy. Electronic address: ana.lleo@humanitas.it.
authors.forename: Ana
authors.initials: A
authors.lastname: Lleo
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood*; D001324:Autoantigens / Q000276:immunology; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000276:immunology*; D015209:Cholangitis, Sclerosing / Q000175:diagnosis / Q000276:immunology*; D051047:Dihydrolipoyllysine-Residue Acetyltransferase / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000276:immunology*; D024101:Mitochondrial Proteins / Q000276:immunology
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D001324:Autoantigens; D024101:Mitochondrial Proteins; C052359:DLAT protein, human; D051047:Dihydrolipoyllysine-Residue Acetyltransferase
keywords: Autoantibody; Biliary tree; Cholestatic diseases; Laboratory tools; Mitochondria; Nuclear proteins