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Investigations

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

Liver function tests:

  • raised serum alkaline phosphatase
  • raised serum aspartate aminotransferase in 90% of cases
  • raised serum bilirubin levels in many patients

Other features:

  • hypergammaglobulinaemia occurs in one third; IgM levels increased in 50% of cases
  • raised hepatic copper levels in nearly 90% of cases
  • autoantibodies are less frequent than in autoimmune chronic active hepatitis and primary biliary cirrhosis:
    • smooth muscle antibodies occur in 11% of cases
    • P-ANCA positive in more than 50% of cases
    • antinuclear antibodies in 6 to 35% of cases
    • antimitochondrial antibodies almost never occur

Liver biopsy may reveal:

  • a fibrous obliterating cholangitis
  • loss of interlobular and adjacent septal bile ducts

ERCP may demonstrate:

  • multiple annular strictures, separated by round or slightly dilated duct segments
  • the intrahepatic and extrahepatic bile ducts have a beaded appearance

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