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Primary Biliary Cirrhosis: Clinical Aspects

  • Marshall M. Kaplan

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.

Keywords

Primary Biliary Cirrhosis Renal Tubular Acidosis Wilson Disease Primary Biliary Cirrhosis Patient Prospective Control Trial 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Plenum Publishing Corporation 1989

Authors and Affiliations

  • Marshall M. Kaplan
    • 1
    • 2
  1. 1.Division of GastroenterologyTufts University School of MedicineNew EnglandUSA
  2. 2.Medical Center HospitalsBostonUSA

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