The clinical course of primary sclerosing cholangitis (PSC) is highly variable. Some patients are asymptomatic for years, whereas others develop end-stage liver disease or cholangiocarcinoma (CCA) shortly after diagnosis.1–4 Previous studies have reported a median transplant-free survival between 12 and 21 years1,2 and a cumulative 10-year risk of CCA of 6%–11%.2,3 PSC is typically associated with inflammatory bowel disease (IBD), most commonly ulcerative colitis (UC). Recent reports have demonstrated a more benign clinical course of the disease in patients with Crohn’s disease (CD) compared with those with UC.