Primary sclerosing cholangitis (PSC) is a chronic disease that slowly damages the bile ducts inside and outside the liver. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the gall bladder and the small intestine, where it helps digest fats and fatty vitamins.
In those affected with PSC, the bile ducts become blocked due to inflammation and scarring. This causes bile to accumulate in the liver, where it gradually damages liver cells and causes cirrhosis, or scarring of the liver. As cirrhosis progresses and the amount of scar tissue in the liver increases, the liver slowly loses its ability to function. The scar tissue may block drainage of the bile ducts leading to infection of the bile.
PSC advances very slowly. Many patients may have the disease for years before symptoms develop. Symptoms may remain at a stable level, they may come and go, or they may progress gradually. Liver failure may occur 10-15 years after diagnosis, but this may take even longer for some PSC patients. Many people with PSC will ultimately need a liver transplant, typically about 10 years after being diagnosed with the disease..
The exact cause of PSC is not known. About 70 percent of patients are men. It may be related to bacterial or viral infections, as well as problems in the immune system. Genetic factors may also play a role. PSC was once thought to be an uncommon disease, but recent studies suggest that it may be more common than previously thought.
There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery may be useful to temporarily improve bile flow.