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Disease pathophysiology:
PSC is a progressive disease, often leading to biliary cirrhosis within 10 to 15 years. Patients who are asymptomatic at the time of diagnosis fare better than those who are symptomatic, but the disease tends to progress in either case. The average overall survival time is approximately 10 years from the date of diagnosis.
Disease statistics:
Cholangiocarcinoma is a dreaded complication of PSC, occurring in 4% to 20% of patients; the incidence is even higher in autopsy studies. The development of cholangiocarcinoma is often accompanied by clinical decline but can be difficult to diagnose, even when it is suspected, because of the low sensitivity of biliary brush cytology in this setting.
Disease treatment:
Up to 20% of patients with PSC develop jaundice, cholangitis, or both, caused by a dominant stricture of the biliary tree, which can be treated with balloon dilation with or without the placement of a biliary stent. This is usually done endoscopically, but it can be done percutaneously. Although there are no established guidelines for surveillance for cholangiocarcinoma in patients with PSC, a high index of suspicion should be maintained.