At diagnosis, the majority of patients are asymptomatic and present e.g. for workup of elevated serum levels of AP or cholesterol [55, 56] . In symptomatic patients, fatigue and pruritus are the most common complaints and have been reported in 21% and 19% of patients at presentation, respectively [27, 57] . Unexplained discomfort in the right upper quadrant of the abdomen has been reported in approximately 10% of patients [58] . In the majority of asymptomatic and untreated patients, overt symptoms develop within 2 to 4 years, although one third may remain symptom-free for many years [27, 56] .