A chronic liver disease of unknown origin marked by inflammation and obliteration of the intrahepatic and extrahepatic bile ducts. The disease progresses silently and steadily and in most patients leads to cirrhosis, portal hypertension, and liver failure. Seventy percent of patients are men and the mean age at diagnosis is 39. Liver transplantation can be used to treat patients who develop cirrhosis from this disease.
A chronic condition characterized by a presumed failure of the immune system to recognize the cells comprising the bile ducts as self, leading to their targeted destruction. The exact cause of this disease remains uncertain, although it is commonly associated with another inflammatory disorder of the digestive tract known as ulcerative colitis. Over time, the inflammation of the ducts results in the formation of scar tissue, leading to the development of multiple constricted areas (strictures) that impede the flow of bile and promote bacterial infection. Liver transplantation offers the most promising prospects for long-term survival.