title: Characterization and clinical impact of antinuclear antibodies in primary biliary cirrhosis.
abstract: OBJECTIVE
  The clinical impact of antinuclear antibodies in primary biliary cirrhosis is uncertain. We analyzed in detail the antinuclear antibodies reactivity of primary biliary cirrhosis patients and correlated the fine specificities observed with clinical, biochemical, and immunologic parameters.
  
  
  METHODS
  A total of 96 consecutive primary biliary cirrhosis patients and 283 pathologic controls were studied. To dissect the fine antinuclear antibodies specificities we used different techniques, such as indirect immunofluorescence on cryostat tissue sections and cell culture (HEp-2 cells), counterimmunoelectrophoresis with thymus and spleen extracts, ELISA assays with recombinant Sp100 and purified gp210 and Lamin B receptor, and immunoblot with several recombinant nuclear and cytoplasmic antigens.
  
  
  RESULTS
  Antinuclear antibodies were detected in 53% of patients, with the following hierarchy of specificities: 27% anti-Sp100, 16% "multiple nuclear dots," 16% anti-gp210, 16% anti-centromere, 7% XR1, 6% anti-lamin B receptor, 5% anti-SS-A/Ro, 5% anti-ribonucleoprotein, 4% XR2, 2% anti-SS-B/La, 2% perinuclear antineutrophil cytoplasmic antibodies, and 1% anti-double-stranded deoxyribonucleic acid. Several patients showed multiple specificities. The "multiple nuclear dots" pattern was detected more often in antimitochondrial antibodies negative patients. In particular, primary biliary cirrhosis specific antinuclear antibodies (anti-Sp100, anti-gp210, and anti-lamin B receptor) were detected in nine of 13 antimitochondrial negative primary biliary cirrhosis cases. Anti-gp210 was more frequent in patients with more pronounced cholestasis and more impaired liver function.
  
  
  CONCLUSIONS
  Antinuclear antibodies reactivities are present in more than half of primary biliary cirrhosis patients and target diverse autoantigens located in distinct subnuclear structures. Anti-gp210 identifies a subgroup of primary biliary cirrhosis patients with more serious liver disease. Positivity for anti-Sp100, anti-gp210, and anti-lamin B receptor, either alone or in combination, may act as a serologic marker of antimitochondrial antibodies negative primary biliary cirrhosis.
authors.affiliation: Dipartimento di Medicina Interna, Cardioangiologia, Epatologia, Alma Mater Studiorum, Università di Bologna, Policlinico S. Orsola-Malpighi, Italy.
authors.forename: Paolo
authors.initials: P
authors.lastname: Muratori
authors.forename: Luigi
authors.initials: L
authors.lastname: Muratori
authors.forename: Rodolfo
authors.initials: R
authors.lastname: Ferrari
authors.forename: Fabio
authors.initials: F
authors.lastname: Cassani
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Bianchi
authors.forename: Marco
authors.initials: M
authors.lastname: Lenzi
authors.forename: Luis
authors.initials: L
authors.lastname: Rodrigo
authors.forename: Antonio
authors.initials: A
authors.lastname: Linares
authors.forename: Dolores
authors.initials: D
authors.lastname: Fuentes
authors.forename: Francesco B
authors.initials: FB
authors.lastname: Bianchi
mesh_terms: D000974:Antibodies, Antinuclear / Q000032:analysis*; D000918:Antibody Specificity; D003378:Counterimmunoelectrophoresis; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D015151:Immunoblotting; D008105:Liver Cirrhosis, Biliary / Q000276:immunology*; D008297:Male; D008875:Middle Aged
chemical_list: D000974:Antibodies, Antinuclear