Incidence, Prevalence, Medication Use, and Transplant Rates in Paediatric Autoimmune Hepatitis—A Nationwide Cohort Study

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BACKGROUND AND AIMS: Despite the aggressive nature of paediatric autoimmune hepatitis (P-AIH), population-based epidemiology is poorly described. We aimed to validate an algorithm to identify patients with P-AIH in the Danish National Patient Registry (DNPR) and report incidence rates, prevalence, and describe medication exposure and liver transplantation rates. METHODS: This was a nationwide, register-based study. We used a smaller population-based P-AIH cohort as true positives for validation. The best-performing algorithm was then used to identify all patients in Denmark with P-AIH (1978-2022). Incidence rates and prevalence for the period 1996-2022 are presented. We used the National Prescription Register and DNPR to report on the use of prednisolone, thiopurine, and tacrolimus as well as liver transplantation. RESULTS: Based on the best-performing algorithm (sensitivity 0.94, specificity 1.0), we identified 222 incident patients. The incidence rate rose from 0.7/100000 person-years in 1999-2001 to the highest observed incidence rate in 2014-2016 (4.5/100000 person-years). The prevalence rose from 2.2/100.000 persons in 1999-2001 to the highest observed in the 2017-2019 period (8.8/100.000 persons). During the first year, 212 (95%) were treated with prednisolone, 159 (72%) with thiopurines, and 23 (10%) with tacrolimus. After five years, 219 (99%) had been treated with prednisolone, 178 (80%) with thiopurines, and 44 (20%) with tacrolimus. Native liver survival was 97% and 93% at 5 and 10 years after diagnosis. CONCLUSION: The incidence and prevalence of paediatric autoimmune hepatitis in Denmark have increased over the past two decades. Most patients receive corticosteroids and thiopurines, and native liver survival remains high.

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