Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease of the hepatobiliary system, characterized by pro-gressive inflammation, fibrosis, and stricturing of the intraand extra-hepatic bile ducts. [1][2][3] Histopathologically, PSC is characterized by inflammation and fibrosis of the intra-and extra-hepatic ductal epithelium, which can ultimately progress to end-stage liver disease and cirrhosis, and increase the risk for cholangiocarcinoma. 4,5 The presentation of PSC is highly variable, ranging from asymptomatic in the early stages, to recurrent episodes of cholangitis with fever, jaundice, pruritus, abdominal pain and fatigue. However, median survival tends to be significantly decreased in patients who are symptomatic at the time of diagnosis. 6,7 Epidemiological studies of PSC have reported a prevalence rate of approximately 1 per 10,000, and incidence rates between 0.4 and 2.0 per 100,000 per year in Northern Europe and the US. 8 PSC conforms to the definition of rare diseases, affecting less than 200,000 individuals in the