Primary biliary cirrhosis - Definition

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Primary biliary cirrhosis is a rare disease, probably of autoimmune origin, which leads to the destruction of the bile ducts in the liver. In most cases, it is middle-aged women who are affected. Primary biliary cirrhosis initially appears as itching and yellowing of the skin (jaundice) associated with the increase in the volume of the liver (hepatomegaly) and spleen (splenomegaly). The progression takes a few years and leads to a general deterioration of health, bleeding, and liver failure, which causes the patient's death. The treatments use a molecule, ursodeoxycholic acid, or liver transplantation.