primary sclerosing cholangitis. Also called idiopathic sclerosing cholangitis. A chronic (long term) liver disorder where the ducts carrying bile from the liver to the intestine, and often the ducts carrying bile within the liver, become inflamed, thickened, scarred (sclerotic), and blocked. PSC can occur with other disorders, including inflammatory bowel disease or by itself. PSC often causes jaundice (yellowing of the skin and eyes), generalized itching all over the body, upper abdominal pain, and infection. In time it progresses to cirrhosis of the liver (irreversible scarring) and liver failure, requiring liver transplantation. Diagnosis is based on physical exam, routine lab tests, and is confirmed by special radiological tests called cholangiography (ERCP, percutaneous cholangiogram, MRCP). Prognosis depends on the age of the person, degree of jaundice, the stage of PSC and the size of the spleen. Many patients die within 10 years of diagnosis unless a liver transplant is performed.
Primary Sclerosing Cholangitis. An irritation or scarring of the bile ducts located outside of the liver (extrahepatic bile ducts) caused by the immune system. This disease usually does not affect the small bile ducts within the liver. In patients with ulcerative colitis, an increased incidence of this disease has been identified.