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Current Perspectives in Hepatology pp 157–169Cite as

Primary Biliary Cirrhosis: Clinical Aspects

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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 is 20–80 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; i. e., the alkaline phosphatase and γ-glutamyltranspeptidase are disproportionately elevated compared with other biochemical tests. A positive antimitochondrial antibody test, present in 95% of patients with primary biliary cirrhosis, makes the diagnosis very likely. It is still important to ascertain that the bile ducts are patent. This can usually be done with ultrasonography or computed tomography (CT) scanning. If the results of these tests are equivocal, endoscopic retrograde cholangiopancreatography should be done. Percutaneous needle biopsy of the liver will provide confirmatory information and permit histologic staging of the disease. This is helpful in assessing prognosis.

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Authors and Affiliations

  1. Division of Gastroenterology, Tufts University School of Medicine, New England, USA

    Marshall M. Kaplan

  2. Medical Center Hospitals, Boston, Massachusetts, 02111, USA

    Marshall M. Kaplan

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Editors and Affiliations

  1. Division of Gastroenterology/Hepatology, Veterans Administration Medical Center, USA

    Leonard B. Seeff M.D. (Chief, Professor of Medicine) (Chief, Professor of Medicine)

  2. Georgetown University School of Medicine, Washington, D.C., USA

    Leonard B. Seeff M.D. (Chief, Professor of Medicine) (Chief, Professor of Medicine)

  3. Division of Gastroenterology, Georgetown University School of Medicine, Washington, D.C., USA

    James H. Lewis M.D., F.A.C.P., F.A.C.G. (Associate Professor of Medicine) (Associate Professor of Medicine)

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© 1989 Plenum Publishing Corporation

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Kaplan, M.M. (1989). Primary Biliary Cirrhosis: Clinical Aspects. In: Seeff, L.B., Lewis, J.H. (eds) Current Perspectives in Hepatology. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-7041-3_14

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