30% rise due in primary sclerosing cholangitis prevalence by 2040

Study spanned 18 countries in Europe, North America, Asia-Pacific, Australasia

Lindsey Shapiro, PhD avatar

by Lindsey Shapiro, PhD |

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The global prevalence of primary sclerosing cholangitis (PSC), or the total number of existing cases, is expected to increase substantially over the next several years, according to recent projections.

Pooled study data suggest the highest prevalence of PSC was seen in the Australasia region and the lowest in high-income areas of the Asia-Pacific. Accordingly, the most dramatic increases are expected in Australasia in the coming years. A lack of data from certain parts of the world means it’s difficult to truly estimate the global PSC burden, however.

“Our findings highlight the urgent need for enhanced surveillance and research into PSC, particularly in regions where its impact may be escalating,” the scientists wrote. The results were described in the study, “The epidemiological trends and projected future of primary sclerosing cholangitis by 2040: An updated meta-analysis and modeling study,” which was published in PLOS One.

PSC is an autoimmune disease that causes chronic cholangitis, that is, inflammation in the tubes that carry the digestive fluid bile from the liver to the intestines. Progressive scarring and damage can lead to irreversible liver damage or liver failure, and related symptoms.

The disease occurs about two times more often in men than women and is most commonly diagnosed between ages 30-40. Its prevalence and incidence, that is, the rate of new cases within a specific time period, vary by geographical region, but the highest rates are generally reported in Northern Europe, with lower rates seen in Asia. Most research on the disease’s epidemiology come from small studies of European and North American populations, meaning better global estimates are needed, the researchers said.

“Such predictions can aid in the development of public health initiatives aimed at alleviating the global burdens associated with PSC,” they wrote.

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Tracking incidence, prevalence of PSC

To provide updated estimates of PSC rates globally and in specific regions, the scientists reviewed PSC epidemiological studies published up to 2024. A total of 29 studies, published from 1970 to 2024, were analyzed. They spanned 18 countries in four geographical regions — Western Europe, high-income North America, high-income Asia-Pacific, and Australasia.

The overall worldwide pooled incidence rate of PSC across these studies was 0.65 per 100,000 people and the prevalence was 7.52 per 100,000 people. Australasia had the highest incidence at 0.92 per 100,000 people and prevalence at 13.94 per 100,000 people, while Asia-Pacific had the lowest incidence and prevalence (0.21 and 1.6 per 100,000 people, respectively).

The pooled mortality rate of people with PSC was 0.1 per 100,000 people, with the lowest seen in the high-income Asia-Pacific and the highest in North America.

The data also varied by individual countries, although a lack of available data made evaluating individual Asia-Pacific countries difficult.

Future rates of PSC

The researchers then developed a model to predict PSC prevalence from 2024 to 2040. According to their projections, the global PSC prevalence is due to significantly increase, by nearly 30% from 17.9 to 22.98 cases per 100,000. The most dramatic increase is predicted in Australasia, where the prevalence is expected to grow by 40.84%. This is followed by Western Europe, where a 28.79% rise is anticipated, with a 21.12% jump in the U.K. alone.

Less pronounced increases are projected for Asia-Pacific (1.4%) and North America (5.5%), with rates predicted to rise by 10% in the U.S. and nearly 16% in Canada.

A growing burden

“This sharp upward trend highlights the growing burden of PSC on healthcare systems worldwide and underscores the importance of advancing research, early diagnosis, and treatment strategies to mitigate its impact in the coming decades,” wrote the researchers, who said the exact factors underlying the observed geographical differences aren’t established, but could include genetic factors, lifestyle, diet, or sun exposure, among others.

“Continued investigation into the underlying factors contributing to these geographic disparities will be essential for developing targeted prevention and treatment strategies,” wrote the researchers, who emphasized that limitations in the data mean the findings should be interpreted with caution.

The lack of data in certain regions, including South America and Africa, hampers progress in truly understanding the global PSC burden. Differences in healthcare access, diagnostic processes, and other factors across regions can also complicate the matter.

“Future research should prioritize conducting large-scale, population-based studies in diverse geographic regions, with particular focus on underrepresented areas,” the researchers wrote. “By doing so, we can advance our understanding of PSC and its impact on global health, ultimately facilitating more effective prevention and treatment strategies.”