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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 .
Cholestatic pruritus is the sensation of itch due to nearly any liver disease, but the most commonly associated entities are primary biliary cholangitis, primary sclerosing cholangitis, obstructive choledocholithiasis, carcinoma of the bile duct, cholestasis (also see drug-induced pruritus), and chronic hepatitis C viral infection and other forms of viral hepatitis.
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 (PBC), also known as primary biliary cirrhosis, is an auto-immune, inflammatory liver disease which produces bile duct injury, fibrosis, cholestasis and eventual cirrhosis. [15] It is much more common in women than men and can cause jaundice, itching and fatigue.
For patients with primary biliary cholangitis, current guidelines recommend about 13–15 mg/kg of ursodeoxycholic acid as a first line treatment. [121] This drug stimulates biliary bicarbonate secretion, improves survival without having to resort to a liver transplantation, and is very well tolerated—making it an ideal treatment. [ 122 ]
In primary biliary cholangitis, there is fibrosis around the bile duct, the presence of granulomas and pooling of bile. [98] Lastly in alcoholic cirrhosis, there is infiltration of the liver with neutrophils. [95] Macroscopically, the liver is initially enlarged, but with the progression of the disease, it becomes smaller.
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 ]
biliary dyskinesia; ICD-10 code K83: other diseases of the biliary tract: cholangitis (including ascending cholangitis and primary sclerosing cholangitis) obstruction, perforation, fistula of biliary tract (bile duct) spasm of sphincter of Oddi; biliary cyst; biliary atresia