MC910216387[1]What is primary sclerosing cholangitis?

Primary Sclerosing Cholangitis (PSC) is a rare liver disease where the bile ducts, which are the passages that carry bile from the liver to the intestines, become narrowed due to inflammation and fibrosis. Over time, scar tissue can build up and block the bile ducts completely. Blockages can occur inside and outside the liver in any part of the biliary tree, and the narrowed or blocked bile ducts disrupt the flow of bile out of the liver. This causes a variety of symptoms, including:

• fatigue
• general aching
• pruritus (itching)
• pain in the upper right side of the abdomen
• jaundice
• chills
• fever

Many people with PSC are asymptomatic, meaning they do not show any outward symptoms to indicate they are living with a chronic illness. The progression of the disease varies highly between patients and the progression of PSC cannot be predicted for the individual patient. Because patients may not have any obvious symptoms, PSC is frequently discovered by accident. Some people can have PSC for years without knowing it.

What causes PSC?

The cause of PSC is still unknown, however, there is a close association with PSC and Inflammatory Bowel Disease (IBD) – around 70% of PSC patients also have IBD. It is not clear how these conditions are linked and whether they share similar causes, but it is believed it may be related to an autoimmune reaction to an infection or toxin in those predisposed to develop PSC.

There are many explanations of PSC.  Here are just a few:

Oslo University Hospital

Studies of Primary Sclerosing Cholangitis (STOPSC)

University of California San Francisco

At present, there is no known cure or treatment to slow the progression of the disease, but some symptoms can be managed.