Autoantibody profile of primary sclerosing cholangitis

Hanan Gur, Guoqiu Shen, Made Sutjita, Jeff Terrberry, Iyad Alosachie, Noori Barka, Hun Chi Lin, James B. Peter, Pier L. Meroni, Marshall Kaplan, Eric Gershwin, Yehuda Shoenfeld*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Primary sclerosing cholangitis (PSC) is a chronic progressive liver disease of unknown etiology. It has been suggested that genetic and immunological factors are important in its pathogenesis. The present study examined the prevalence of 23 different autoantibodies in 25 PSC sera, by ELISA, in order to better define the autoimmune profile of PSC. The results indicate that 88% of PSC patients produced at least 1 autoantibody, and 36% had reactivity to multiple autoantibodies. Moreover, 35% of the PSC patients produced anti-endothelial-cell antibodies (AECA) and 75% of the sera contained perinuclear antineutrophil cytoplasmic antibodies (pANCA), detected by indirect immunoflurescence. The prominent ANCA autoantibody was anti-cathepsin-G, demonstrated in 35% of the patients. The multiplicity of the autoantibody profile, revealed in the present study, points to the autoimmune characteristics of PSC. In addition, the association of ANCA and of AECA in PSC may suggest a pathogenic role for these antibodies in PSC.

Original languageEnglish
Pages (from-to)76-82
Number of pages7
Issue number2
StatePublished - 1995
Externally publishedYes


  • Autoantibodies
  • Autoimmune diseases
  • Primary sclerosing cholangitis


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