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Cholestatic Liver Disease pp 33–50Cite as

Primary Biliary Cirrhosis

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

Abstract

Primary biliary cirrhosis (PBC) is a relatively rare but important cause of chronic cholestatic liver disease that affects predominantly middle-aged women (Talwalkar and Lindor, Lancet 362(9377):53–61, 2003). Small and medium-sized bile ducts are destroyed by an inflammatory process, presumably autoimmune in nature, resulting in cholestasis, advanced fibrosis, cirrhosis, and liver failure if untreated. Most patients are asymptomatic at presentation, likely as a result of widespread use of screening biochemical tests and the specific anti-mitochondrial antibody assay. Symptomatic patients usually present with either fatigue or pruritus. Ursodeoxycholic acid (UDCA) is the only established treatment for PBC and may improve survival in selected patients. However, some patients do not respond adequately to UDCA and might need alternative therapeutic approaches.

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Abbreviations

AASLD:

American Association for the Study of Liver Diseases

ALT:

Alanine aminotransferase

AlkPhos:

Alkaline phosphatase

AMA:

Anti-mitochondrial antibodies

AST:

Aspartate aminotransferase

ELISA:

Enzyme-linked immune assay

FDA:

Food and Drug Administration

LT:

Liver Transplantation

MRS:

Mayo risk score

PBC:

Primary biliary cirrhosis

UDCA:

Ursodeoxycholic acid

ULN:

Upper limits of normal

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  1. Gastroenterology and Hepatology, 10701 East Blvd 111E (W), Cleveland, OH, 44106, USA

    Marina G. Silveira M.D.

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  1. Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, Arizona, USA

    Elizabeth J. Carey

  2. Mayo Clinic, Phoenix, Arizona, USA

    Keith D. Lindor

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Silveira, M.G. (2014). Primary Biliary Cirrhosis. In: Carey, E., Lindor, K. (eds) Cholestatic Liver Disease. Clinical Gastroenterology. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-1013-7_3

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