Autoimmune sclerosing cholangitis: Evidence and open questions

J Autoimmun. 2018 Dec:95:15-25. doi: 10.1016/j.jaut.2018.10.008. Epub 2018 Oct 23.


Juvenile sclerosing cholangitis is a rare chronic hepatobiliary disorder characterized by inflammation of the intra- and/or extrahepatic bile ducts, bile duct dilatation, narrowing and obliteration, and, histologically, by inflammatory bile duct damage leading to periductular fibrosis. The diagnosis is based on endoscopic retrograde cholangiopancreatography or magnetic resonance cholangiopancreatography. In children, it may be associated to a variety of systemic and hepatic conditions: thus, the term "primary" sclerosing cholangitis should be reserved for the rare cases without a known cause. Small duct disease is diagnosed in the presence of histological features diagnostic of sclerosing cholangitis and normal cholangiography. Autoimmune sclerosing cholangitis (ASC) is a form of sclerosing cholangitis with strong autoimmune features overlapping with those of autoimmune hepatitis (AIH). It is a well-recognized nosological entity in paediatrics, where it accounts for the majority of sclerosing cholangitis cases. It is as prevalent as AIH in children, is equally frequent in males and females, half of the patients have concomitant inflammatory bowel disease, virtually all patients have raised immunoglobulin G levels and positive anti-nuclear and/or anti-smooth muscle antibodies. Half of the ASC patients respond well to standard immunosuppressive treatment for AIH with the addition of ursodeoxycholic acid, but the transplant rate is higher than in AIH, and post-transplant recurrence is frequent. A number of open questions remain: are ASC and AIH distinct entities or different manifestations of the same condition? What is the role of histology? Is small duct disease a specific entity? What is the relationship between ASC and adult primary sclerosing cholangitis? What is the role of inflammatory bowel disease? In addition, validated diagnostic criteria for ASC are needed.

Keywords: Autoimmune sclerosing cholangitis; Cholangiography; Inflammatory bowel disease; Sclerosing cholangitis; Small duct disease.

Publication types

  • Review

MeSH terms

  • Adolescent
  • Adult
  • Autoantibodies / biosynthesis*
  • Autoimmune Diseases / complications
  • Autoimmune Diseases / diagnostic imaging
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Bile Ducts / diagnostic imaging
  • Bile Ducts / drug effects
  • Bile Ducts / immunology*
  • Child
  • Cholangiopancreatography, Endoscopic Retrograde
  • Cholangiopancreatography, Magnetic Resonance
  • Cholangitis, Sclerosing / complications
  • Cholangitis, Sclerosing / diagnostic imaging
  • Cholangitis, Sclerosing / drug therapy
  • Cholangitis, Sclerosing / immunology*
  • Clinical Studies as Topic
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Inflammatory Bowel Diseases / complications
  • Inflammatory Bowel Diseases / diagnostic imaging
  • Inflammatory Bowel Diseases / drug therapy
  • Inflammatory Bowel Diseases / immunology*
  • Liver / diagnostic imaging
  • Liver / drug effects
  • Liver / immunology*
  • Male
  • Ursodeoxycholic Acid / therapeutic use


  • Autoantibodies
  • Immunosuppressive Agents
  • Ursodeoxycholic Acid