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Incidence, Prevalence, Medication Use, and Transplant Rates in Paediatric Autoimmune Hepatitis-A Nationwide Cohort
Mikkel Malham1,2,3,4, Sabine Jansson1,2, Fie Brantbjerg Tinning5
1Department of Pediatric and Adolescent Medicine, Copenhagen University Hospital - Amager and Hvidovre, Hvidovre, Denmark.
Summary
Incidence and prevalence of paediatric autoimmune hepatitis (P-AIH) in Denmark have increased significantly. Most children receive corticosteroids and thiopurines, with high native liver survival rates.
Area of Science:
- Hepatology
- Paediatric Gastroenterology
- Epidemiology
Background:
- Paediatric autoimmune hepatitis (P-AIH) is an aggressive liver disease with poorly described population-based epidemiology.
- Accurate identification of P-AIH cases in national registries is crucial for epidemiological studies.
Purpose of the Study:
- To validate an algorithm for identifying P-AIH patients in the Danish National Patient Registry (DNPR).
- To report on the incidence, prevalence, medication exposure, and liver transplantation rates for P-AIH in Denmark.
Main Methods:
- A nationwide, register-based study utilizing the Danish National Patient Registry (DNPR) and National Prescription Register.
- Algorithm validation was performed using a smaller, known P-AIH cohort.
- Data on P-AIH cases were collected from 1978 to 2022, with incidence and prevalence reported for 1996-2022.
Main Results:
- A validated algorithm with 0.94 sensitivity and 1.0 specificity identified 222 incident P-AIH patients.
- Incidence rose from 0.7/100,000 person-years (1999-2001) to 4.5/100,000 person-years (2014-2016).
- Prevalence increased from 2.2/100,000 persons (1999-2001) to 8.8/100,000 persons (2017-2019).
- High rates of prednisolone (95%) and thiopurine (72%) use were observed within the first year of diagnosis, increasing to 99% and 80% respectively after five years.
- Native liver survival was high at 97% (5 years) and 93% (10 years).
Conclusions:
- The incidence and prevalence of paediatric autoimmune hepatitis in Denmark have significantly increased over the past two decades.
- Corticosteroids and thiopurines are the primary medications used in P-AIH management.
- Native liver survival rates in paediatric patients with autoimmune hepatitis remain high.
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