Serum autoantibodies in patients with primary sclerosing cholangitis

Paul Angulo, James B. Peter, M. Eric Gershwin, Carolee K. DeSotel, Yehuda Shoenfeld, Alaa E.E. Ahmed, Keith D. Lindor

Research output: Contribution to journalArticlepeer-review

151 Scopus citations


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 ≥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 auto antibodies 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.

Original languageEnglish (US)
Pages (from-to)182-187
Number of pages6
JournalJournal of Hepatology
Issue number2
StatePublished - Feb 2000
Externally publishedYes


  • Autoantibodies
  • Inflammatory bowel diseases
  • Mayo risk score
  • Primary sclerosing cholangitis

ASJC Scopus subject areas

  • Hepatology


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