Primary biliary cirrhosis: Is there still a place for histological evaluation?

摘要

Primary biliary cirrhosis (PBC) is a chronic cholestatic disease that mainly affects middle-aged women and naturally progresses towards cirrhosis and liver failure. It is a well established indication of liver transplantation (IT). The disease is characteristically associated with antimitochon-drial antibodies (AMA), which makes it an archetype of autoimmune liver disease. The primary hepatic lesion is a granulomatous chronic non-suppurative destructive cholan-gitis that specifically targets small intrahepatic (interlobular and septal) bile ducts and progressively results in biliary duc-topenia and cirrhosis. This typical lesion classically observes a segmental, non-uniform distribution within the liver, so that it is classically lacking in half to two-thirds of needle biopsy specimens. It is also rarely seen in the most advanced stages of the disease when extensive fibrotic scars profoundly disturb the architecture of the liver, making interlobular bile ducts hardly identifiable.