title: Antinuclear antibodies as ancillary markers in primary biliary cirrhosis.
abstract: Antimitochondrial antibodies are the serological hallmark of primary biliary cirrhosis (PBC). Besides antimitochondrial antibodies, the autoantibody profile of PBC includes antinuclear antibodies (ANA) which are detectable by indirect immunofluorescence in up to 50% of PBC patients. Two immunofluorescence patterns are considered 'PBC-specific': the multiple nuclear dots and rim-like/membranous patterns. The target antigens of the multiple nuclear dots pattern have been identified as Sp100 and promyelocytic leukemia protein, whereas the rim-like/membranous pattern is given by autoantibodies recognizing multiple proteins such as gp210, nucleoporin p62 and the lamin B receptor. Other ANA, especially those already known in the rheumatological setting, such as anticentromere, anti-SSA/Ro and anti-dsDNA antibodies, can be frequently found in PBC, often coexisting in the same patient. In this article, we will report on recent progress in the antigenic characterization of ANA in PBC, their detection with both traditional assays and Western blot/ELISA with molecularly defined nuclear antigens, and we will discuss their clinical significance.
authors: Department of Clinical Medicine, Alma Mater Studiorum-University of Bologna, S.Orsola-Malpighi Hospital, Italy. alessandro.granito@unibo.it
authors: Alessandro
authors: A
authors: Granito
authors: Paolo
authors: P
authors: Muratori
authors: Chiara
authors: C
authors: Quarneti
authors: Georgios
authors: G
authors: Pappas
authors: Ronny
authors: R
authors: Cicola
authors: Luigi
authors: L
authors: Muratori
mesh_terms: D000974:Antibodies, Antinuclear / Q000276:immunology*; D034961:Antigens, Nuclear / Q000276:immunology; D001324:Autoantigens / Q000276:immunology; D015415:Biomarkers / Q000378:metabolism; D015153:Blotting, Western / Q000379:methods; D004797:Enzyme-Linked Immunosorbent Assay / Q000379:methods; D018604:Epitope Mapping; D019084:Fluorescent Antibody Technique, Indirect / Q000379:methods; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000175:diagnosis / Q000276:immunology; D008562:Membrane Glycoproteins / Q000276:immunology; D028861:Nuclear Pore Complex Proteins / Q000276:immunology; D009687:Nuclear Proteins / Q000276:immunology; D011379:Prognosis; D000071156:Promyelocytic Leukemia Protein; D018160:Receptors, Cytoplasmic and Nuclear / Q000276:immunology; D014157:Transcription Factors / Q000276:immunology; D025521:Tumor Suppressor Proteins / Q000276:immunology; D000097682:Lamin B Receptor
chemical_list: D000974:Antibodies, Antinuclear; D034961:Antigens, Nuclear; D001324:Autoantigens; D015415:Biomarkers; D008562:Membrane Glycoproteins; D028861:Nuclear Pore Complex Proteins; D009687:Nuclear Proteins; D000071156:Promyelocytic Leukemia Protein; D018160:Receptors, Cytoplasmic and Nuclear; D014157:Transcription Factors; D025521:Tumor Suppressor Proteins; D000097682:Lamin B Receptor; C063865:NUP210 protein, human; C049312:nuclear pore protein p62; C066642:SP100 protein, human; C070576:PML protein, human