Primary sclerosing cholangitis is a disease in which the bile ducts inside and outside the liver become narrower due to inflammation and scarring. This causes bile to accumulate in the liver and can result in damage to liver cells.
Although primary sclerosing cholangitis has been considered a rare disease, recent studies suggest that it is more common than previously thought. It may occur alone, but is often associated with inflammatory diseases of the colon, such as chronic ulcerative colitis.
Primary sclerosing cholangitis appears to be more common in men than women. Initially, many individuals have no symptoms and the disease is detected because of abnormal lab test results. It usually begins In the 30’s, 40’s and 50’s, and is commonly associated with itching, fatigue and jaundice. Episodes of fever, shaking and chills can be frequent distressing symptoms.
The course of the disease is unpredictable and not well understood. Patients may have the disease for many years before symptoms develop. Symptoms may then persist at a stable level or progress. Sometimes, primary sclerosing cholangitis can even cause liver failure and death. Bile duct malignancy may have similar symptoms, so an accurate diagnosis is important.
The cause of primary sclerosing cholangitis remains unknown. There is no evidence that it is inherited. Hepatitis viruses are not associated with It. As primary sclerosing cholangitis is often associated with inflammatory bowel disease, there may be a common cause for both. More research is needed to find the cause and develop effective treatment.
Presently, there is no specific treatment for primary sclerosing cholangitis. Research is being done on various medications. Symptoms may be treated with varying success by antibiotics, vitamins and medications to control itching. In some Instances, surgery may be effective in relieving Jaundice and other symptoms. When medical treatment is not helpful and there is severe liver failure, liver transplantation may be indicated. Liver transplantations have a survival rate of 60 percent or more. The slow progression of the disease makes it possible to plan for the transplant over many months.