Alagille Syndrome
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Environmental Agents May Trigger Autoimmune Liver Disease

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Environmental agents may play a role in the development of autoimmune diseases, specifically primary biliary cholangitis (PBC), autoimmune hepatitis (AIH), and primary sclerosing cholangitis (PSC), according to results from a large population-based study presented at The International Liver Congress 2019.

The cause of autoimmune liver diseases is not known although a link between a genetic predisposition to autoimmunity and environmental factors has been suggested. Disease clustering of PBC in 2 areas of Northern and England and New York have been previous been reported.

The study identified a cohort from North-East England and North Cumbria who had PBC (n = 2150), AIH (n = 963), or PSC (n = 472). Postal addresses and year of diagnosis were used for spatial point analyses and spatio-temporal analyses.

Significant spatial clustering was noted for all 3 diseases at approximately 1 to 2 km. Evidence for additional clustering for AIH and PSC at approximately 10 km and 7.5 km in PBC.

“This study suggests that exposure to a persistent, low-level environmental agent may have played a role in the pathogenesis of all three autoimmune liver diseases studied, not just PBC,” said Dr Jessica Dyson, Associate Clinical Lecturer, Newcastle University and Consultant Hepatologist at Newcastle upon Tyne Hospitals NHS Foundation Trust.


Dyson J, Shirley M, Blain A, et al. Disease clustering in autoimmune liver diseases points towards environmental factors being important in their aetiology. EASL 2019; Abstract PS-014.