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Primary biliary cirrhosis, also known as primary biliary cholangitis (PBC), is an autoimmune disease of the liver. It is marked by slow progressive destruction of the small bile ducts of the liver, with the intralobular ducts and the Canals of Hering (intrahepatic ductules) affected early in the disease. When these ducts are damaged, bile and other toxins build up in the liver (cholestasis) and over time damages the liver tissue in combination with ongoing immune related damage. This can lead to scarring, fibrosis and cirrhosis. PBC is a relatively rare disease, with some studies showing that it affects up to 1 in 3–4,000 people. The sex ratio is at least 9:1 female to male.〔 As cirrhosis is only a feature of advanced disease, a change of name to "primary biliary cholangitis" was proposed by patient advocacy groups in 2014.〔(【引用サイトリンク】title=PBC Name Change Press Release )〕〔(【引用サイトリンク】title=Primary Biliary Cirrhosis Name Change Initiative )〕 Use of the new name was advocated in the medical literature in September 2015.〔 ==Signs and symptoms== People with PBC experience fatigue (80%) that leads to sleepiness during the daytime; more half of those have severe fatigue. 20–70% have itching.〔 Those with more severe disease may have jaundice (yellowing of the eyes and skin). PBC impairs bone density and there is an increased risk of fracture.〔 Xanthelasma (skin lesions around the eyes) or other xanthoma may be present as a result of increased cholesterol levels. PBC can eventually progress to cirrhosis of the liver. This in turn may lead to a number of symptoms or complications: * Fluid retention in the abdomen (ascites) in more advanced disease * Enlarged spleen in more advanced disease * Oesophageal varices in more advanced disease * Hepatic encephalopathy, including coma in extreme cases in more advanced disease. People with PBC may have the findings of an associated extrahepatic autoimmune disorder such as rheumatoid arthritis or Sjögren's syndrome (in up to 80% of cases).〔〔 抄文引用元・出典: フリー百科事典『 ウィキペディア（Wikipedia）』 ■ウィキペディアで「primary biliary cirrhosis」の詳細全文を読む スポンサード リンク
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