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Primary sclerosing cholangitis (PSC) definition
Primary sclerosing cholangitis describes a disease process in which the bile
ducts in the liver become inflamed, narrow and prevent bile from flowing
The liver produces bile to help digest food in the intestine. Bile from liver
cells is transported through the bile ducts in the biliary tree, where it then
enters the gallbladder. When food enters the small intestine, bile helps break
down fat into fatty acids so that they can be absorbed and used by the body.
This also helps in the absorption of fat soluble vitamins (A,D,E & K).
As the bile ducts become inflamed and narrow, bile cannot easily flow and
begins to back up. This increases the pressure within the liver causing liver
cells to become inflamed. Over time, this inflammation decreases blood flow
within the liver, increasing the pressure in the portal vein. This eventually
causes portal hypertension, a back up in the portal system causing veins that
line the esophagus, stomach, and intestine to swell (varices) and the
As the disease progress, liver cells die and are replace by scar tissue. This
is called cirrhosis and is associated with liver failure.