title: Serum immunological profile in patients with chronic autoimmune cholestasis.
abstract: OBJECTIVE
  To compare patients who had biochemical and histological features of chronic autoimmune cholestasis (CAIC) using serological autoantibody profiling.
  
  
  METHODS
  Patients (n = 174 CAIC; 79 AMA(-) and 95 AMA(+)) were profiled for the following antibodies: antinuclear antibodies (ANAs), antimitochondrial antibodies (AMAs), antismooth muscle actin (SMA, mainly F-actin), antiperinuclear cytoplasmic neutrophil antibodies (pANCAs), anti-SP100, anti-GP210, anti-M2 (2-oxo-acid dehydrogenase complexes), and antisoluble liver antigen (SLA). Liver specimens were reviewed according to staging, biliary interface activity, lobular hepatitis, granulomas, cholestasis, and florid ductal lesion.
  
  
  RESULTS
  In patients who were AMA(-) by indirect immunofluorescence (IIF), 34.6% were positive for anti-M2 by immunoblotting. In 49 definitively AMA(-) patients, 24 (48.9%) showed ANA-primary biliary cirrhosis (PBC)-related antibodies (rim-like, multiple nuclear dots, anti-SP100, or anti-GP210). There were no differences in immunological, biochemical, or histopathological features between IIF-AMA(+) patients and AMA(-) patients with anti-M2 or ANA-PBC-related antibodies. AIH-related autoantibodies were found in 13 patients (7.5%). Patients with AMAs or ANA-PBC-related antibodies had higher IgM levels, whereas patients with antibodies highly specific for AIH had higher AST, bilirubin, and IgG levels, and AIH scores, and higher grades of lobular hepatitis. Overall, three distinct categories of patients were observed: AMA(+) or AMA(-) patients with ANA-PBC-related antibodies; AMA(-) patients with non-PBC-related ANAs; and patients with AIH-related antibodies together with serum PBC markers.
  
  
  CONCLUSIONS
  Since these three groups had immunological, biochemical, and histopathological differences, they ought to be considered as separate clinical subentities rather than as merely AMA(-) or AMA(+) patients with autoimmune cholestasis.
authors.affiliation: Unidad de Hepatología, Hospital Universitario de Valme, Sevilla, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Romero-Gómez
authors.forename: Ingeborg
authors.initials: I
authors.lastname: Wichmann
authors.forename: Javier
authors.initials: J
authors.lastname: Crespo
authors.forename: Albert
authors.initials: A
authors.lastname: Parés
authors.forename: Luis
authors.initials: L
authors.lastname: Rodrigo
authors.forename: Antonia
authors.initials: A
authors.lastname: Alvarez
authors.forename: Moisés
authors.initials: M
authors.lastname: Diago
authors.forename: Fernando
authors.initials: F
authors.lastname: Pons-Romero
authors.forename: Diego
authors.initials: D
authors.lastname: Sanchez-Munoz
authors.forename: José
authors.initials: J
authors.lastname: Aguilar-Reina
authors.forename: Raúl J
authors.initials: RJ
authors.lastname: Andrade
authors.forename: Javier
authors.initials: J
authors.lastname: Salmeron
authors.forename: Pilar
authors.initials: P
authors.lastname: Sánchez-Pobre
authors.forename: Jaime M
authors.initials: JM
authors.lastname: Rebollo
authors.forename: Rafael
authors.initials: R
authors.lastname: Martin-Vivaldi
authors.forename: Victor
authors.initials: V
authors.lastname: Castellano-Megias
authors.forename: Antonio
authors.initials: A
authors.lastname: Nuñez-Roldan
authors.forename: Miquel
authors.initials: M
authors.lastname: Bruguera
mesh_terms: D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000276:immunology* / Q000473:pathology; D002779:Cholestasis / Q000276:immunology* / Q000473:pathology; D002908:Chronic Disease; D005260:Female; D006801:Humans; D008099:Liver / Q000473:pathology; D008297:Male; D008875:Middle Aged; D008928:Mitochondria / Q000276:immunology
chemical_list: D001323:Autoantibodies