Primary Biliary Cirrhosis (PBC) is a chronic disease of unknown cause, affecting women as much as 10 to 20 times as frequently as men and is perhaps the only autoimmune disease never reported in children; its incidence gradually rises with age. It is characterized by chronic intrahepatic cholestasis due to chronic inflammation and necrosis of intrahepatic bile ducts and progresses insidiously to biliary cirrhosis.
Its prevalence has been estimated to be 2.3-14.4 per 100,000. The histologic abnormalities in the liver have been divided into four stages. The earliest changes (stage I) are most specific and consist of localized areas of infiltration of intrahepatic bile ducts with lymphocytes and necrosis of biliary epithelial cells. In stage II there is proliferation of bile ductules, prominent infiltration of portal areas with lymphoid cells, and early portal fibrosis.
Stage II is characterized by reduction of the inflammatory changes, paucity of bile ducts in the portal triads, and increased portal fibrosis. In stage IV, fibrosis is prominent in biliary cirrhosis and a marked increase in hepatic copper is found. Thus, the pathologic process is characterized by slowly progressive, spotty destruction of bile ducts, with associated inflammation and fibrosis and, ultimately, cirrhosis.