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Primary sclerosing cholangitis (PSC) occurs when bile ducts become blocked due to inflammation or scarring, which causes bile to accumulate in the liver. Over time, it damages liver cells and can lead to cirrhosis. When that happens, the liver loses its ability to function.
Bile ducts are the conduit for transferring bile from the liver and gallbladder through the pancreas to the small intestine.
PSC is commonly a slow-growing disease. Liver failure may occur 10-15 years after diagnosis and many people with the disease will ultimately need a liver transplant. PSC may also lead to bile duct cancer.
Here is what you need to know about PSC.
Many people with PSC do not get symptoms, especially in the early stages of the disease. When symptoms do occur the most common are fatigue, pruritus, or itching of the skin, and jaundice, a yellowing of the skin and eyes. These symptoms may come and go, but they may worsen over time. As the disease continues, the bile ducts may become infected, which can lead to episodes of fever, chills and abdominal pain.
The cause of this disease 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 is considered an uncommon disease, but recent studies suggest that it may be more common than previously thought.
Because many PSC patients have no symptoms, the disease is often discovered through abnormal results on routine liver blood tests. Formal diagnosis is usually made by cholangiography, an X-ray test involving injection of dye into the bile ducts, or by an MRI.
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 when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow.
Many PSC patients suffer a gradual loss of liver function. If liver failure becomes severe, a liver transplant may be necessary. The outcome for patients with PSC who have undergone transplantation is excellent. The survival rate for two or more years is about 80 percent, with a good quality of life after recovery
PSC is slightly more common in men than women. It usually affects people between the ages of 30 and 60. As many as 75 percent of patients with PSC also have inflammatory bowel diseases, usually ulcerative colitis.
PSC has been known for 100 years, but now doctors are able to diagnose it very early. This means that treatment can begin before the liver is severely damaged. Scientists are continuing to study the disease to find the cause and understand its development.
In addition, drug therapy trials, involving a large number of patients around the world, are exploring the potential use of several additional medications to lessen the symptoms and control liver damage.
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