investigations

Last reviewed 01/2018

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