Primary sclerosing cholangitis is an inflammation of the bile ducts caused by inflammation, narrowing and blocking of the bile ducts in the liver. This increases pressure in the liver over time, causing damage to liver cells, reducing blood flow in the liver and increasing pressure in the portal vein. As the disease progresses, liver cells die and are replaced by scar tissue, eventually leading to cirrhosis due to primary sclerosing cholangitis.

Sclerosing cholangitis is a chronic, slowly progressive, sometimes asymptomatic disease. People with long-term sclerosing cholangitis can lead to liver damage, liver failure due to fibrosis.