Sometimes, large fluctuations in serum bilirubin concentration can occur, even early in the disease, possibly as a result of transient bile duct obstruction or superimposed bacterial cholangitis. Early in the course of the disease, the serum albumin concentration and prothrombin time are normal. Later, when cirrhosis develops, the albumin concentration falls and the prothrombin time can be prolonged. The characteristic findings include multifocal strictures and dilatations of the intrahepatic and extrahepatic bile ducts. Liver biopsy is usually confirmatory but not diagnostic.
Liver biopsy is also important in determining if the patient has cirrhosis.
These causes of secondary sclerosing cholangitis can usually be ruled-out by history, physical examination and laboratory tests. PSC is a progressive disease that leads to cirrhosis and liver failure. The median survival, or time to liver transplantation, is around ten years from the time of diagnosis. The course of disease is usually complicated by recurrent bouts of superimposed bacterial cholangitis. A very high serum bilirubin concentration, complications of cirrhosis, older age and anemia indicate a poor prognosis and suggest that the patients should be referred for orthotopic liver transplantation.