Clinical features

Over 70% of patients are men, the majority of which are less than 45 years of age.

Fatigue represents the most frequently reported symptom, affecting up to 71% of patients, and tends to worsen as the disease progresses, peaking in the afternoon and evening. Right upper quadrant (RUQ) abdominal pain occurs in 20% to 40% of patients, while pruritus affects approximately 38%, often intensifying in the evening and during winter months.

Other symptoms are those associated with chronic liver disease, for example, weight loss, ascites, jaundice, variceal bleeding and hepatic encephalopathy.

Hepatomegaly is found in about 50% of patients; less often, there is splenomegaly, hyperpigmentation and xanthomas.

Reference

1. Tabibian JH, Bowlus CL. Primary sclerosing cholangitis: A review and update. Liver Res. 2017 Dec;1(4):221-230