The Epidemiology of Pediatric Autoimmune Hepatitis in Scotland: A National Cohort Study

Harry Sutton; Rachel Tayler; Iain Chalmers; Jennifer Cowieson; Karen Fraser; Paul Henderson; Richard Hansen

doi:10.1097/PG9.0000000000000223

The Epidemiology of Pediatric Autoimmune Hepatitis in Scotland: A National Cohort Study

JPGN Rep. 2022 Jul 29;3(3):e223. doi: 10.1097/PG9.0000000000000223. eCollection 2022 Aug.

Authors

Harry Sutton¹, Rachel Tayler¹, Iain Chalmers², Jennifer Cowieson¹, Karen Fraser¹, Paul Henderson^{3

4}, Richard Hansen¹

Affiliations

¹ From the Department of Paediatric Gastroenterology, Royal Hospital for Children, Glasgow, United Kingdom.
² Department of Paediatric Gastroenterology, Royal Aberdeen Children's Hospital, Aberdeen, United Kingdom.
³ Department of Paediatric Gastroenterology and Nutrition, Royal Hospital for Children and Young People, Edinburgh, United Kingdom.
⁴ Child Life and Health, University of Edinburgh, Edinburgh, United Kingdom.

Abstract

Autoimmune hepatitis (AIH) is a rare, but potentially severe, cause of liver disease in children. We aimed to summarize how children with AIH in Scotland presented, were investigated and managed in addition to producing novel epidemiological data and outcomes.

Methods: All prevalent pediatric patients with AIH cared for in pediatric services between January 2013 and September 2018 were included. Individual patient data were obtained from electronic patient records in the 3-main academic pediatric centers in Scotland covering the entire population.

Results: Thirty-eight patients were included (25 female) with median follow-up of 33 months (range, 2-145 mo) and 136 total patient years. The incidence between 2014 and 2017 was 0.49/100 000/y (95% confidence interval, 0.29-0.78) and point prevalence between 2013 and 2018 was 1.75/100 000 (95% confidence interval, 1.42-2.13). Thirty-five (92%) patients were autoantibody positive, most commonly anti-nuclear antibody (63%) and anti-smooth muscle antibody (42%). Thirty-seven (97%) patients had induction therapy with oral corticosteroids, 30 (79%) required maintenance treatment with azathioprine, and 23 (61%) received ursodeoxycholic acid. There were 1.4 disease flares per 10 patient years and 3 patients required liver transplantation with an overall 5-year survival rate without the need for transplantation of 95%.

Conclusions: We calculated a novel incidence and prevalence rate for pediatric AIH in Scotland. Nearly all were invariably treated initially with corticosteroids with most placed-on azathioprine as maintenance therapy. Outcomes were generally favorable with low rates of disease flares and the need for transplantation being rare.

Keywords: autoimmune hepatitis; azathioprine; corticosteroids; liver transplant; prevalence.

Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition.