Curbside Consultation of the Liver: 49 Clinical Questions

Mitchell L. Shiffman, MD

Question 31: When Should I Initiate Treatment With Ursodeoxycholic Acid And What Dose Should I Utilize In My Patient With Primary Biliary Cirrhosis? (continued)

Puneet Puri, MD; Velimir A. Luketic, MD

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease that primarily affects middle-aged women. The progressive injury in PBC is a 2-step process that begins with immune-mediated destruction of the microscopic intrahepatic bile ducts and subsequent retention of bile constituents, including bile salts, within the liver. Bile salts are powerful detergents that damage cells both by directly disrupting cell and organelle membranes and by activating intracellular signals promoting apoptosis. In PBC, the result of cholangiocyte and hepatocyte injury is self-perpetuating liver…