Evidence for an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis

J Hepatol. 1996 Jun;24(6):699-705. doi: 10.1016/s0168-8278(96)80266-2.


Background/aims: Autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis are chronic liver diseases with probable autoimmune background. Overlapping features have been described for primary biliary cirrhosis and autoimmune hepatitis. In contrast, there have been only a few case reports on an overlap of autoimmune hepatitis and primary sclerosing cholangitis.

Methods: We describe three male patients with clinical and histological overlapping features of primary sclerosing cholangitis and autoimmune hepatitis.

Results: All initially asymptomatic patients had elevated levels of aminotransferases, alkaline phosphatase, gamma-glutamyltranspeptidase and IgG. Anti-nuclear antibodies and/or smooth muscle antibodies were positive and anti-neutrophil cytoplasmic antibodies were detected in all patients. Retrograde endoscopic cholangiography showed bile-duct strictures characteristic for primary sclerosing cholangitis. Histopathology showed necro-inflammatory activity of portal tracts with bridging necrosis in all patients at the time of first diagnosis. Aminotransferase levels and the necro-inflammatory activity responded well to immunosuppressive treatment. Predominant periductular fibrosis as a typical histopathological feature of primary sclerosing cirrhosis was seen to develop in all patients. Cholestatic serum parameters remained elevated and periductular fibrosis as endoscopic bile duct changes progressed despite immunosuppression.

Conclusions: We suggest that these patients present an overlap syndrome of autoimmune hepatitis and primary sclerosing cholangitis as they fulfill the diagnostic criteria for both conditions.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Adult
  • Antibodies, Antinuclear / blood
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / diagnosis*
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology
  • Biopsy
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangitis, Sclerosing / blood
  • Cholangitis, Sclerosing / complications
  • Cholangitis, Sclerosing / diagnosis*
  • Cholangitis, Sclerosing / drug therapy
  • Diagnosis, Differential
  • Follow-Up Studies
  • Hepatitis, Chronic / blood
  • Hepatitis, Chronic / complications
  • Hepatitis, Chronic / diagnosis*
  • Hepatitis, Chronic / drug therapy
  • Humans
  • Immunoglobulin G / blood
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Syndrome
  • Transaminases / blood


  • Antibodies, Antinuclear
  • Immunoglobulin G
  • Immunosuppressive Agents
  • Transaminases