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The Epidemiology of Pediatric Autoimmune Hepatitis in Scotland: A National Cohort Study
Harry Sutton1, Rachel Tayler1, Iain Chalmers2
1From the Department of Paediatric Gastroenterology, Royal Hospital for Children, Glasgow, United Kingdom.
Insights
Autoimmune hepatitis in children is rare but serious. This study found favorable outcomes in Scotland with corticosteroid and azathioprine treatment, and low rates of liver transplantation.
Area of Science:
- Pediatric Hepatology
- Autoimmune Diseases
- Epidemiology
Background:
- Autoimmune hepatitis (AIH) is a rare but severe liver disease in children.
- Understanding its presentation, management, and outcomes in pediatric populations is crucial.
Purpose of the Study:
- To summarize the presentation, investigation, and management of pediatric AIH in Scotland.
- To generate novel epidemiological data and assess outcomes for pediatric AIH in Scotland.
Main Methods:
- Retrospective analysis of all prevalent pediatric AIH patients in Scotland (2013-2018).
- Data collected from electronic patient records across three major academic pediatric centers.
- Inclusion of all patients receiving care within pediatric services during the study period.
Main Results:
- Calculated incidence (0.49/100,000/year) and prevalence (1.75/100,000).
- 92% of 38 patients were autoantibody positive; 97% received corticosteroid induction therapy.
- 5-year survival without transplantation was 95%, with rare disease flares and transplant needs.
Conclusions:
- Established novel incidence and prevalence rates for pediatric AIH in Scotland.
- Corticosteroids and azathioprine are standard initial and maintenance therapies.
- Pediatric AIH in Scotland demonstrates generally favorable outcomes with effective management.
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.
