Primary Sclerosing Cholangitis (PSC) is a disease that causes inflammation and subsequent destruction of the bile ducts, the small tubes that drain bile from the liver. The condition can affect the bile ducts within the liver and the ducts that drain the liver and gallbladder into the intestine. It is a chronic and progressive disease which eventually leads to cirrhosis of the liver. Patients with Primary Sclerosing Cholangitis often also have inflammatory bowel disease.
Patients with PSC often present with jaundice or yellowing of the eyes and skin. The accumulation of bile salts may cause itching. Ineffective drainage of the liver can result in recurrent episodes of infection called cholangitis. Eventually, cirrhosis may cause complications such as confusion, abdominal swelling or gastrointestinal bleeding.
The diagnosis of PSC is often suspected initially when liver enzymes in the blood are elevated. The diagnosis is confirmed by performing a test to visualize the bile ducts, such as an MRI or ERCP. A liver biopsy may be performed to assess the degree of damage that has been done to the liver.
No specific therapy has been shown to cure or delay the progression of PSC to cirrhosis or liver failure. Episodes of infection are treated with antibiotics. Strictures, or narrowing of the bile ducts, may be treated with dilatation or placement of a stent, a plastic tube that is placed in the bile duct to bypass the area of obstruction. Ultimately, patients with PSC will require liver transplantation.