Primary biliary cirrhosis is a slowly progressive autoimmunedisease of the liver that primarily affects women. Its peakincidence occurs in the fifth decade of life, and it is uncommonin persons under 25 years of age. Histopathologically, primarybiliary cirrhosis is characterized by portal inflammation andimmune-mediated destruction of the intrahepatic bile ducts.These changes occur at different rates and with varying degreesof severity in different patients. The loss of bile ducts leadsto decreased bile secretion and the retention of toxic substanceswithin the liver, resulting in further hepatic damage, fibrosis,cirrhosis, and eventually, liver failure.
From the Division of Gastroenterology, TuftsNew England Medical Center, Boston (M.M.K.); and the Division of Rheumatology, Allergy and Clinical Immunology, School of Medicine, University of California at Davis, Davis (M.E.G.).
Address reprint requests to Dr. Kaplan at the Division of Gastroenterology, TuftsNew England Medical Center, 750 Washington St., Boston, MA 02111, or at mkaplan@tufts-nemc.org.
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Primary Biliary Cirrhosis
Brancatelli G., Federle M. P., Blachar A., Gong Y., Christensen E., Gluud C., Roblin X., Bonaz B., Kaplan M. M., Gershwin M. E.
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N Engl J Med 2005;
353:2719-2720, Dec 22, 2005.
Correspondence
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