Serum autoantibodies in patients with primary sclerosing cholangitis

J Hepatol. 2000 Feb;32(2):182-7. doi: 10.1016/s0168-8278(00)80061-6.


Background/aim: Primary sclerosing cholangitis is a chronic cholestatic syndrome with a presumed autoimmune basis frequently associated with inflammatory bowel disease. The aim of this study was to determine the profile and significance of serum autoantibodies in patients with primary sclerosing cholangitis.

Methods: Serum samples taken from 73 untreated patients (32 female and 41 male, median age 45 years) with well-defined primary sclerosing cholangitis, and from 75 healthy age- and sex-matched controls were assayed for 20 different autoantibodies.

Results: Of 73 patients, 71 (97%) were positive for at least 1 autoantibody; whereas 59/73 patients (81%) were positive for > or =3 antibodies. Patients with primary sclerosing cholangitis had a significantly greater rate of positivity than controls for antinuclear, anticardiolipin, antineutrophil cytoplasmic, and antithyroperoxidase antibodies as well as rheumatoid factor. The rate of positivity and serum levels of any of these 20 autoantibodies were not significantly different between patients with primary sclerosing cholangitis and inflammatory bowel disease and those without inflammatory bowel disease. Anticardiolipins were the single group of antibodies that had a significant correlation with the Mayo risk score (r=0.49, p<0.001) and histologic stage of disease (r=0.30, p<0.01).

Conclusions: Primary sclerosing cholangitis is associated with a high proportion of non-organ specific autoantibodies. Anticardiolipin antibodies appear to be related to the severity of primary sclerosing cholangitis and may be a useful prognostic marker.

MeSH terms

  • Adult
  • Antibodies, Anticardiolipin / blood
  • Antibodies, Antineutrophil Cytoplasmic / blood
  • Antibodies, Antinuclear / blood
  • Autoantibodies / blood*
  • Cholangitis, Sclerosing / blood
  • Cholangitis, Sclerosing / chemically induced
  • Cholangitis, Sclerosing / immunology*
  • Female
  • Humans
  • Inflammatory Bowel Diseases / complications
  • Iodide Peroxidase / immunology
  • Male
  • Middle Aged
  • Reference Values
  • Rheumatoid Factor / blood
  • Risk Factors


  • Antibodies, Anticardiolipin
  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Antinuclear
  • Autoantibodies
  • Rheumatoid Factor
  • Iodide Peroxidase