Background and Aims: Although the incidence and prevalence of primary sclerosing cholangitis (PSC) vary by location, global studies on its geographic distribution and future trends are lacking. Herin, we projected the further prevalence of PSC across the world from 2019 to 2040 using an illness-death multi-state model.

Methods: We performed a comprehensive systematic search across various databases to locate all primary studies published until 2023 that provided data on the incidence rate, and prevalence of PSC, as well as mortality rates in each region. Then, we developed an illness-death model using available data to predict the future prevalence of PSC that covers the years from 2020 to 2040.

Results: Spain and Italy have the lowest incidence rate of PSC at 0.04 and 0.1 cases per 100 000 people, respectively, while Finland has the highest incidence rate at 1.58 cases per 100 000 people. We projected that the prevalence of PSC in 2040 will experience a significant surge of 92.3% in the Asia-Pacific region, 90.5% in North America, 83.2% in Western Europe, and 59.5% in Australia. In 2040, Spain and Italy are projected to witness a significant surge in the prevalence of PSC, with a staggering 176.4% and 150% rise, respectively.

Conclusions: Our findings indicate a substantial rise in the number of individuals affected by PSC in recent years and estimate a significant future burden of the disease.