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Gastroenterol Clin North Am. 2011 Jun;40(2):373-86, viii. doi: 10.1016/j.gtc.2011.03.008. Epub 2011 Apr 13.

Primary biliary cirrhosis.

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Division of Gastroenterology & Hepatology, Drexel University College of Medicine, 219 North Broad Street, Fifth Floor, Philadelphia, PA 19107, USA.


Primary biliary cirrhosis is a chronic autoimmune inflammatory disease of the liver with a striking female preponderance. It has an insidious onset and typically affects middle-aged women. The disease manifests gradually with symptoms of fatigue, pruritis, and increased alkaline phosphatase levels on laboratory evaluation. The hallmark of the disease is the circulating antimitochondrial antibody. Histology is characterized by inflammation of the bile ducts, destruction of cholangiocytes, and subsequent cholestasis, progressing to biliary cirrhosis. The standard treatment for primary biliary cirrhosis is ursodeoxycholic acid, which improves survival, but the disease can still lead to cirrhosis and liver failure over decades.

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