| [ Summary ] |
Primary biliary cirrhosis (PBC), a chronic cholestatic liver disease, which is characterized by the destruction and disappearance of intrahepatic small bile ducts. Middle aged women are those primarily affected by PBC. Autoimmune mechanisms may be involved in the process of bile duct destruction. Long standing cholestasis accompanied with varying degrees of interface hepatitis may eventually develop into liver cirrhosis. Anti-mitochondrial antibodies (AMA), which are directed to the E2 component of the 2-oxo acid dehydrogenase complex, are positive in more than 90 % of PBC patients. They are a highly sensitive and specific marker for the diagnosis of PBC. PBC is diagnosed based on three major findings including 1) elevation of biliary enzymes such as ALP and γ-GTP, 2) positive AMA, and 3)characteristic pathological findings including CNSDC, granulomas, bile duct loss or chronic cholestasis. Diagnosis of PBC may be difficult in cases assosiated with AMA-negative PBC or PBC-autoimmune hepatitis (AIH) overlap syndrome. Clinical stages are classified into asymptomatic PBC (aPBC) and symptomatic PBC (sPBC). The latter is further classified into s1-PBC with itching, unaccompanied by jaundice and s2-PBC with jaundice. |