The bile ducts within and/or outside the liver become narrowed and scarred. The cause is unknown. The disease is associated with inflammatory bowel disease, and genetic factors may play a causative role. The disease is progressive and most patients develop biliary cirrhosis, persistent jaundice, and liver failure. The diagnosis is made, in part, by eliminating other diseases of the biliary system (for example, by absence of stones in the gallbladder or biliary system). The disease occurs more often in men. This disorder is rare in children.