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A trio of rare, autoimmune liver diseases are more prevalent in the Faroe Islands compared to the rest of the world.¹

A team, led by Kári Rubek Nielsen, Medical Department, National Hospital of the Faroe Islands, Torshavn, Faroe Islands Center of Health Science, Faculty of Health Science and Nursing, University of the Faroe Islands, assessed the incidence of 3 rate autoimmune liver diseases.

A Lack of Data

There are currently not many population-based studies focusing on the epidemiology of autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis are uncommon.

The rates of autoimmune hepatitis appear to be significantly higher in Europe, especially in Scandinavian countries, compared to the rest of the world. The prevalence of this disease is 1-2 per 100,000 people per year worldwide, between 5-25 per 100,000 population in European countries and the Asia-Pacific region and 24 per 100,000 people per year in Finland.

For primary biliary cholangitis, the global incidence is 1-2 per 100,000 people per year, with a variation from 0.23-4.3 per 100,000 population per year and a prevalence range of 1.9-4.34 per 100,000 population.

The prevalence of primary sclerosing cholangitis ranges from 0.0-2.0 per 100,000 per year.

The 10-year morality of autoimmune hepatitis ranges from 23-32%, the 10-year mortality of primary biliary cholangitis ranges from 41-62%, and the 10-year mortality of primary sclerosing cholangitis ranges from 8-15%.

“The incidence and prevalence of autoimmune liver diseases vary, but in general, there seems to be a higher incidence and prevalence of autoimmune liver diseases in the Nordic Countries,” the authors wrote.

Liver Disease in the Faroe Islands

In the nationwide registry-based cohort study, the investigators examined all cases of autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis in the Faroe Islands between 2004-2021 and searched all medical records to assess diagnostic criteria and cause of death.

The results show an incidence of autoimmune hepatitis of 5.2 per 100,000 people per year. In addition, the incidence of primary biliary cholangitis was 2.5 per 100,000 people per year and the incidence of primary sclerosing cholangitis was 0.7 per 100,000 people per year.

The point prevalence per 100,000 people at the conclusion of the study was 71.8 for autoimmune hepatitis, 38.5 for primary biliary cholangitis, and 11.0 for primary sclerosing cholangitis. A total of 9 patients with autoimmune hepatitis died following a median of 3 years. Of this group, 3 died of hepatocellular carcinoma (HCC) and 2 died from liver failure.

Moreover, 5 patients with primary biliary cholangitis died after a median of 7 years, 1 with HCC and 1 because of liver failure. Finally 1 patients with primary sclerosing cholangitis died of cholangiocarcinoma.

“The incidence and prevalence of AIH, PBC and PSC in the Faroe Islands are among the highest reported in population-based settings,” the authors wrote.

References:

Nielsen, K. R., Midjord, J., Johannesen, H. L., & Grønbæk, H. (2023). A nationwide study of autoimmune liver diseases in the Faroe Islands: Incidence, prevalence, and causes of death 2004 - 2021. International Journal of Circumpolar Health, 82(1). https://doi.org/10.1080/22423982.2023.2221368