Primary Sclerosing Cholangitis (PSC) is a disease in which the immune system damages the large bile ducts within the liver. Bile ducts are tubes within the liver which drain bile into the gallbladder and intestine. In this disease the large bile ducts develop strictures and narrowing which prevent bile from flowing into the gallbladder and intestine. This causes bile to “back-up” into the liver. This damages liver cells and leads to increasing scaring within the liver and eventually cirrhosis. This process takes approximately 10-20 years.
PSC is not contagious. Patients inherit a genetic tendency to develop this disease and many persons with PSC have family members with other immune disorders. Patients with PSC frequently have another immune disorder, Ulcerative Colitis or Crohns Disease.
Many persons with PSC are asymptomatic. However, as the disease progresses and the strictures within the bile ducts become more severe many persons develop itching, jaundice and fatigue.
PSC causes the blood liver enzyme tests to be abnormal. A specific antibody may also be present in the blood of patients with PSC. Patients with PSC may also have elevated blood cholesterol.
Unfortunately, no treatment has been shown to be effective for PSC. Although URSO is effective in another bile duct disease, PBC, several studies have shown that USO is not effective in PSC. In fact, a recent study has demonstrated that URSO may make patients with PSC worse. The only effective treatment for patients with advanced PSC is liver transplant. Patients with PSC should therefore be monitored at regular intervals and offered liver transplant when the disease has progressed to a certain point.
The most severe complication of PSC is bile duct cancer, also called cholangiocarcinoma. It is extremely difficult to identify when bile duct cancer has developed in patients with PSC. Screening tests to identify bile duct cancer early are not very effective. Unfortunately, there is also no good treatment for bile duct cancer. Chemotherapy, radiation or surgery has not been shown to improve survival in patients with bile duct cancer.
Dr. Shiffman and the staff at the Liver Institute of Virginia have extensive experience in the evaluation and management of patients with PSC and can help educate patients about this disease and when a liver transplant should be considered.