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Primary Biliary Cirrhosis

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Book cover Cholestatic Liver Disease

Part of the book series: Clinical Gastroenterology ((CG))

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Abstract

Primary biliary cirrhosis is a chronic progressive cholestatic liver disease that primarily targets middle-aged women. The pathogenesis is unknown, but several lines of evidence suggest genetic and environmental factors initiate an autoimmune process. Routine laboratory testing has led to the earlier detection of disease, so patients are often asymptomatic at the time of diagnosis. Initial symptoms are usually fatigue and pruritus. Signs of decompensated liver disease may eventually develop. Ursodeoxycholic acid is the only recommended treatment and may improve survival in selected patients. Prognostic models help predict natural history of the disease. Liver transplantation is the only definitive therapy once end-stage liver disease occurs.

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  1. University of Florida, Gainesville, FL

    Virginia C. Clark MD & Cynthia Levy MD

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  1. Mayo Clinic, Rochester, MN

    Keith D. Lindor MD  & Jayant A. Talwalkar MD  & 

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Clark, V.C., Levy, C. (2008). Primary Biliary Cirrhosis. In: Lindor, K.D., Talwalkar, J.A. (eds) Cholestatic Liver Disease. Clinical Gastroenterology. Humana Press. https://doi.org/10.1007/978-1-59745-118-5_3

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