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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 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.
In October 2024, the National Institute for Health and Care Excellence (NICE) adopted a recommendation for elafibranor for the treatment of adults with the primary biliary cholangitis based results from the phase 3 ELATIVE trial, in which 51% of patients receiving elafibranor in combination with ursodeoxycholic acid achieved a cholestasis ...
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 ]
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).
Periportal lesions, also known as interface hepatitis, that spare the biliary tree (may include centrizonal necrosis). Bile duct abnormalities (cholangitis, ductal injury, ductular reaction) can be seen and should prompt evaluation for primary biliary cholangitis or sarcoidosis if granulomas are observed.
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.
Ursodeoxycholic acid (UDCA), also known as ursodiol, is a secondary bile acid, produced in humans and most other species from metabolism by intestinal bacteria.It is synthesized in the liver in some species, and was first identified in bile of bears of genus Ursus, from which its name derived. [8]
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