Primary Sclerosing Cholangitis (PSC) is a rare liver disease that is associated with damage to the bile ducts inside and outside the liver. With PSC, bile ducts become inflamed, often leading to scarring with narrowing of the bile ducts. Over time, blockages may occur and bile is trapped in the liver, leading to liver damage and eventually liver failure. Individuals with PSC may eventually require liver transplantation.
PSC often occurs in association with a variety of autoimmune diseases and inflammatory conditions, most notably inflammatory bowel disease (IBD).
In addition, because PSC often occurs in young people, it may inflict a heavy toll on their quality of life and may be associated with a substantial emotional burden.
Currently, there is no effective medical therapy for PSC despite numerous clinical studies conducted over decades. Presently, liver transplantation is the treatment of choice for end-stage PSC. However, PSC can sometimes recur in the transplanted liver, possibly requiring re-transplantation.