Primary biliary cholangitis is a chronic cholestatic disease that is diagnosed based on cholestatic laboratory features, antimitochondrial antibody positivity, or a typical histological pattern. Most patients are asymptomatic at the time of diagnosis.
Pruritus and fatigue are the most common symptoms in symptomatic patients. Ursodeoxycholic acid is the first-line therapy.
If this fails, the prognosis is unfavorable and second-line therapy can be considered. The associated symptoms must be treated.
In advanced disease, liver transplantation should be considered and the complication of portal hypertension must be treated.