Primary Biliary Cirrhosis: Pathology
The essential morphologic feature of primary biliary cirrhosis (PBC) comprises a chronic nonsuppurative destructive cholangitis.1 This is the primary lesion and results in a progressive disappearing bile duct syndrome. Although there is clinical and/or biochemical evidence of a cholestatic syndrome from the time of diagnosis, morphologic evidence of cholestasis does not appear until there has been extensive loss of intrahepatic bile ducts; jaundice with bilirubinostasis is often a still later feature and of poor prognostic import.2 In my view, the progress of the liver injury in PBC is secondary to cholestasis resulting from the loss of bile ducts and is, in effect, a secondary biliary cirrhosis. This chapter describes the morphologic features of PBC, discusses some of the immunological aspects, speculates as to pathogenetic mechanisms, and poses some unanswered questions relating to this enigmatic disease.
KeywordsBile Duct Primary Biliary Cirrhosis Primary Sclerosing Cholangitis Chronic Active Hepatitis Intrahepatic Bile Duct
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