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Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. [ 1 ] [ 2 ] [ 3 ] It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis .
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In primary biliary cholangitis (previously known as primary biliary cirrhosis), the bile ducts become damaged by an autoimmune process. [44] This leads to liver damage. [52] Some people may have no symptoms, while others may present with fatigue, pruritus, or skin hyperpigmentation. [55]
Primary biliary cirrhosis is a serious autoimmune disease of the bile capillaries. [17] Primary sclerosing cholangitis is a serious chronic inflammatory disease of the bile duct, which is believed to be autoimmune in origin. [18] Budd–Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein. [19]
The bile duct scarring that occurs in PSC narrows the ducts of the biliary tree and impedes the flow of bile to the intestines. Eventually, it can lead to cirrhosis of the liver and liver failure. PSC increases the risk of various cancers, including liver cancer, gallbladder carcinoma, colorectal cancer, and cholangiocarcinoma.
Hepatolithiasis is the presence of gallstones in the biliary ducts of the liver. Treatment is usually surgical. It is rare in Western countries, but prevalent in East Asia. [1] The gallstones are normally found proximal to the left and right hepatic ducts. The causes of the disease are poorly understood, but it is suspected that genetics, diets ...
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