Investigations
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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