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Primary biliary cirrhosis 

Primary biliary cirrhosis

Primary biliary cirrhosis

M.F. Bassendine

and D.E.J. Jones


Treatment—discussion of response criteria or UDCA therapy, with need for targeting of second line treatments at non-responders.

A relevant case history from Oxford Case Histories in Gastroenterology and Hepatology has been added to this chapter.

Updated on 27 Feb 2014. The previous version of this content can be found here.
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date: 29 April 2017

Primary biliary cirrhosis is a chronic, cholestatic liver disease in which the biliary epithelial cells lining the small intrahepatic bile ducts are the target for immune-mediated damage leading to progressive ductopenia. The cause is unknown, but presumed to be autoimmune.

The disorder affects women (>90% of cases) and usually has an insidious onset in middle age. Fatigue and pruritus are the most common presenting symptoms. Findings on examination vary widely, ranging from no abnormality to jaundice with hyperpigmentation, scratch marks, and signs of long-standing cholestasis.

Diagnosis of primary biliary cirrhosis is based on three criteria: (1) cholestatic liver function tests, with increases in serum alkaline phosphatase and γ‎-glutamyl transferase, but only modest changes in transaminases; (2) presence of serum antimitochondrial antibodies (AMA), which are found in more than 95% of cases; and (3) compatible liver histology. Many asymptomatic patients are recognized following the incidental discovery of AMA or elevated levels of serum alkaline phosphatase.

Treatment with ursodeoxycholic acid can lead to significant improvement in liver biochemical values and is recommended for some patients. Cholestyramine is used to treat pruritus. No immunosuppressive drug regimen has been proven to be effective. Progression may be slow, but eventually patients can develop cirrhosis, and death may occur from liver failure or complications of cirrhosis such as bleeding oesophageal varices. The disease at one time was a leading indication for liver transplantation, but earlier diagnosis and treatment has now improved the prognosis for many patients.

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