Autoimmune cholangitis and primary biliary cirrhosis - An autoimmune enigma

Hideki Kinoshita, Katsuhisa Omagari, Senga Whittingham, Yuji Kato, Hiromi Ishibashi, Kazuhiro Sugi, Michitami Yano, Shigeru Kohno, Yasuni Nakanuma, Edward Penner, Jozefa Wesierska-Gadek, Sandra Reynoso-Paz, M. Eric Gershwin, John Anderson, Jennifer A. Jois, Ian R. Mackay

Research output: Contribution to journalArticlepeer-review

71 Scopus citations


Aims/Background: Autoimmune cholangitis/cholangiopathy (AIC) is an enigmatic disease marked by chronic cholangitis, antinuclear antibodies (ANA) and sero-negativity for conventionally detected antimitochondrial antibodies (AMA). We examined whether AIC is a distinct entity, an AMA-negative variant of primary biliary cirrhosis (PBC), or a cholangiopathic variant of autoimmune hepatitis (AIH) by comparing the clinical, laboratory and autoantibody profiles of 21 cases of AIC, 37 cases of PBC and 16 cases of AIH from selected Japanese patients. Methods: The specificities of AMA and ANA were determined by immunofluorescence, immunoblotting and enzyme inhibition assays using various mitochondrial and nuclear autoantigens, and the frequencies for these groups were compared. Results: By clinical, biochemical and histological data, AIC and PBC were similar and both were clearly distinct from AIH. Serologically, by immunofluorescence of AMA and ANA, there was polarisation. By immunoblotting, and notwithstanding the negative test for AMA, a proportion of the AIC sera reacted with the E2 subunits of the 2-oxo-acid dehydrogenase enzyme complexes, but more particularly with the lower molecular weight E2 subunits. The antinuclear reactivity in AIC was with centromere, Sp100 and nuclear pore complex proteins as in PBC, but preferentially with the nuclear pore complex. Conclusion: Our results demonstrate that AIC and PBC are similar diseases. However this duo is of interest because, usually, among sets of autoimmune syndromes, differences in serological targetting are matched by differences in clinical presentation: AIC and PBC are an exception to this.

Original languageEnglish (US)
Pages (from-to)122-128
Number of pages7
Issue number2
StatePublished - 1999


  • Anti-gp210
  • Anti-p62
  • Anti-Sp100
  • Antimitochondrial antibodies
  • Antinuclear antibodies
  • Autoimmune cholangitis
  • Autoimmune hepatitis
  • Primary biliary cirrhosis

ASJC Scopus subject areas

  • Hepatology


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