Primary Biliary Cirrhosis

Abstract

PRIMARY biliary cirrhosis, a chronic, progressive, and often fatal cholestatic liver disease, is characterized by the destruction of intrahepatic bile ducts, portal inflammation and scarring, and the eventual development of cirrhosis and liver failure. The diagnosis is usually made in a middle-aged woman (although the age range of patients is 20 to 80 years) who initially has fatigue or itching or who is asymptomatic and has been found to have unexplained hepatomegaly or an elevated serum alkaline phosphatase level. Biochemical tests of liver function typically reveal a cholestatic pattern — that is, the alkaline phosphatase and gamma-glutamyl transpeptidase levels are . . .