Curbside Consultation of the Liver: 49 Clinical Questions

Mitchell L. Shiffman, MD

Question 32: What Type Of Testing Does My Patient With Primary Biliary Cirrhosis Require? (continued)

Velimir A. Luketic, MD

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease characterized by immune-mediated destruction of the small, intrahepatic (intralobular and septal) bile ducts. Loss of bile ducts results in accumulation of bile constituents within the liver that cause hepatocyte injury, fibrosis, and cirrhosis and, ultimately, end-stage liver disease.1 When PBC was first described in the 1850s, cirrhosis was usually already present and the typical patient was a middle-aged woman with itching. Today most patients are asymptomatic at presentation and are identified when an elevated…