Primary sclerosing cholangitis (PSC) is a disease in which the bile ducts inside and outside the liver progressively decrease in size due to inflammation and scarring. As a consequence, bile that is normally carried by these ducts accumulates within the liver. Such obstruction to bile flow can result in damage to liver cells.
Although PSC has been considered a rare disease, recent studies suggest that it is probably more common than originally thought. The disease may occur alone, but frequently exists in association with inflammatory diseases of the colon, such as chronic ulcerative colitis.
Primary sclerosing cholangitis affects both sexes, but may be more common in men. The disease usually begins between ages 30 and 50, and is commonly associated with itching, tiredness, and jaundice. Initially, however, many individuals have no symptoms at all, and the disease becomes apparent because of abnormal blood tests.
The cause of primary sclerosing cholangitis remains unknown. However, there is currently no evidence that the disease is inherited, and viruses known to cause hepatitis have not been associated with primary sclerosing cholangitis. The frequent occurrence of primary sclerosing cholangitis in association with inflammatory bowel disease suggests that a common cause for both diseases may exist. More research is needed to determine the cause of the disease and to develop effective therapy.
Presently, there is no known specific therapy for primary sclerosing cholangitis. Symptoms of sclerosing cholangitis may be treated with varying success by antibiotics, vitamins, and a variety of medications to control itching. In some instances surgery may be effective in alleviating jaundice and other symptoms. Endoscopic therapy is a new method to treat sclerosing cholangitis.
Endoscopic therapy can be performed at the time of ERCP, including sphincterotomy, biliary duct dilatation, nasobiliary perfusion with antibiotics or steroids, or common bile duct stent placement. These methods are generally as effective as surgery in controlling jaundice and other symptoms, and have been shown to improve survival. However, despite adequate medical or surgical therapy, some patients continue to deteriorate because of liver failure. The surgical procedure of last resort in this instance is liver transplantation, which is being performed in centers throughout the United States, with a very good success rate.