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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 .
Primary sclerosing cholangitis is one of the major known risk factors for cholangiocarcinoma, [40] a cancer of the biliary tree, for which the lifetime risk among patients with PSC is 10-15%. [3] This represents a 400-fold greater risk of developing cholangiocarcinoma compared to the general population. [ 1 ]
In primary biliary cholangitis, there is fibrosis around the bile duct, the presence of granulomas and pooling of bile. [100] Lastly in alcoholic cirrhosis, there is infiltration of the liver with neutrophils. [97] Macroscopically, the liver is initially enlarged, but with the progression of the disease, it becomes smaller.
Anti-mitochondrial antibodies (AMA) are autoantibodies, consisting of immunoglobulins formed against mitochondria, [1] primarily the mitochondria in cells of the liver.. The presence of AMA in the blood or serum of a person may be indicative of the presence of, or the potential to develop, the autoimmune disease primary biliary cholangitis (PBC; previously known as primary biliary cirrhosis).
Primary biliary cholangitis (previously known as primary biliary cirrhosis) Primary sclerosing cholangitis; Autoimmune Hepatitis; Other. Right heart failure; Risk factors
T cells recognize biliary epithelial cell antigens causing injury and eventual atresia. Other causes. Primary biliary cirrhosis; Primary sclerosing cholangitis;
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