Primary Sclerosing Cholangitis

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• Disorder of unknown cause characterised by inflammation, fibrosis and strictures of intra- and extrahepatic bile ducts

• Immunological mechanisms implcated

• Associated with UC (80%)


Clinical Features:

• Chronic biliary obstruction and secondary biliary cirrhosis lead to liver failure and death (or transplant) over 10 years


Management:

• Some types respond to corticosteroids

• Colestyramine for pruritus

• UDCA improves cholestasis but no histological effect

• Abx for bacterial choleangitis

• Endoscopic stenting helps symptomatic dominant strictures

• Liver transplantation is indicated in end-stage disease

• Recurrence occurs in 20%

• 5 yr graft survival > 60%