Bile is produced and secreted by the liver cells to aid in the digestion of fats. These compounds are transported through a series of progressively larger tubes that eventually empty into the intestinal tract. In primary sclerosing cholangitis the bile ducts inside and outside of the liver become inflamed and scarred. As the scarring increases, the ducts become blocked. Bile eventually builds up in the liver and damages liver cells. Over extended periods of time, primary scalloping cholangitis may eventually cause liver failure. Primary sclerosing cholangitis has been considered a rare disease, but recent studies suggest that it is more common than previously thought. Although it may occur alone, almost two thirds of the people that develop it will also have some form of inflammatory bowel disease. In particular, ulcerative colitis. The disease usually begins between ages 30 and 60, but it can also arise during childhood. Men are more commonly affected than women.
Causes of Primary Sclerosing Cholangitis
The underlying cause of primary sclerosing cholangitis remains unknown. Current theories as to its etiology involve the possible role of bacteria, viruses, and immune system problems. Primary sclerosing cholangitis is associated with ulcerative colitis, a type of inflammatory bowel disease.
Signs and Symptoms of Primary Sclerosing Cholangitis
Since primary sclerosing cholangitis progresses slowly, a person may have the disease for years before symptoms develop. The primary symptoms are itching, fatigue, and jaundice, which causes yellowing of the eyes or skin. As flow of bile within the network of tubes is slowed, it can lead to infection. This can produce fever, chills abdominal pain, nausea and vomiting.
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Medical Content Last Updated on 07/12/2008
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