Primary Sclerosing Cholangitis

Primary sclerosing cholangitis is a long-term, progressive disease of the liver and gallbladder in which the bile ducts become inflamed and scarred, narrowing the passages that normally carry bile needed for digestion. Its cause is uncertain; researchers suspect an autoimmune process, though the disease does not respond predictably to immune-suppressing drugs, and genetic predisposition, immune dysfunction, and an abnormal makeup of gut bacteria are all thought to contribute. About three-quarters of patients also have inflammatory bowel disease, usually ulcerative colitis, and while nearly half have no symptoms at first and are found only through abnormal liver tests, others develop severe itching, fatigue, jaundice, abdominal pain, and an enlarged liver or spleen, along with recurrent bile duct infections. It substantially raises the risk of bile duct cancer, about 400 times the general population's risk, and median survival from diagnosis to liver transplant or death from the disease is roughly 21 years, though this varies widely by individual case.