Primary biliary cholangitis

Primary biliary cholangitis is a chronic autoimmune liver disease that progressively damages small intrahepatic bile ducts, causing cholestasis and sometimes cirrhosis.

Cholestasis: Impaired bile formation or flow causes bile constituents to accumulate in the liver or bloodstream. Small-duct injury obstructs bile flow, producing the defining physiological disturbance.
Alkaline phosphatase: An enzyme found in several tissues whose blood concentration often rises with bile-duct injury or obstruction. A persistent elevation commonly signals the cholestatic pattern used to detect the disease.
Ursodeoxycholic acid: A hydrophilic bile acid used to improve biochemical markers and outcomes in several cholestatic liver diseases. It is the standard first-line disease-modifying treatment for most diagnosed patients.
Primary sclerosing cholangitis: A chronic cholestatic liver disease marked by inflammation and scarring of intrahepatic and extrahepatic bile ducts. It affects larger ducts and often produces characteristic imaging changes, unlike typical small-duct injury here.
Portal hypertension: Abnormally high pressure in the portal venous system, usually caused by resistance to blood flow through the liver. Advanced fibrosis or cirrhosis can obstruct portal flow and lead to varices or ascites.
Antimitochondrial antibody: An autoantibody directed against components of mitochondrial enzymes, especially the E2 subunit of pyruvate dehydrogenase. It is detectable in most cases and targets an antigen associated with the autoimmune response.
Antinuclear antibody: An autoantibody that binds components of the cell nucleus and is associated with several autoimmune conditions. Certain patterns, including anti-gp210 and anti-sp100, support diagnosis when antimitochondrial antibodies are absent.
Obeticholic acid: A farnesoid X receptor agonist used in some settings to treat cholestatic liver disease. It has been used as an additional treatment when response to ursodeoxycholic acid is inadequate; availability and guidance vary.
Autoimmune hepatitis: A chronic immune-mediated liver disease characterized by hepatocyte inflammation and often elevated aminotransferases and immunoglobulin G. It primarily injures liver cells rather than small bile ducts, though overlap syndromes occur.
Hepatocellular carcinoma: A primary liver cancer arising from hepatocytes, with risk increased by cirrhosis and some chronic liver diseases. Risk rises particularly when the disease has progressed to cirrhosis, guiding surveillance decisions.

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