Primary biliary cirrhosis is a slowly progressive autoimmune disease of the liver that primarily affects women. Its peak incidence occurs in the fifth decade of life, and it is uncommon in persons under 25 years of age. Histopathologically, primary biliary cirrhosis is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. These changes occur at different rates and with varying degrees of severity in different patients. The loss of bile ducts leads to decreased bile secretion and the retention of toxic substances within the liver, resulting in further hepatic damage, fibrosis, cirrhosis, and eventually, liver failure.
Serologically, . . .
http://content.nejm.org/cgi/content/full/353/12/1261
Primary Biliary Cirrhosis
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