Impact of age at diagnosis on disease progression in patients with primary sclerosing cholangitis

Background The median age of diagnosis of primary sclerosing cholangitis (PSC) is ∼30–40 years. Objective We aimed to analyse disease progression and liver‐dependent survival in patients diagnosed with PSC after 50 years of age. Methods Patients with PSC were analysed with regard to their age at diagnosis. Patients with a first diagnosis of PSC after the age of 50 years were considered as the late‐onset group. Results A total of 32/215 (14.9%) patients were diagnosed with PSC after 50 years of age. The proportion of females was significantly higher among patients with late‐onset PSC (48.4 vs. 27.3%; p = 0.02). Patients with later diagnosis required dilatation therapy more often due to dominant stenosis (84.2 vs. 53.1%; p = 0.01) and suffered from recurrent cholangitis more often (48.3 vs. 21.0%; p = 0.003). Patients with late‐onset PSC had reduced transplantation‐free survival (10.5 ± 0.6 years vs. 20.8 ± 1.7 years, p < 0.0001), with progredient liver failure and cholangiocarcinoma as the leading causes of death. Conclusions Patients with later age at diagnosis of PSC displayed a different clinical phenotype with a different sex ratio, immune status and an increased risk for progressive liver failure and biliary malignancies.