How well patients do varies. The disease tends to get worse over time and most patients develop:
Some patients develop recurrent infections of the bile ducts. Some patients have worsening chronic liver disease with ascites and varices.
Patients with this condition have an increased risk of developing cancer of the bile ducts (cholangiocarcinoma), and should be checked on a regular basis (usually each year) with liver scans and blood tests.
Call your health care provider if you have jaundice and itching that do not go away.