PAEDIATRIC autoimmune hepatitis rates have increased in Denmark, with more than a sixfold increase in incidence observed over a 15-year period.
Despite this upward trend, long-term outcomes remain favourable, with high native liver survival reported.
Rising Paediatric Autoimmune Hepatitis Burden in Denmark
Paediatric autoimmune hepatitis is a chronic immune-mediated liver disease in which the body attacks hepatocytes, often requiring prolonged immunosuppressive therapy. Although considered aggressive, population-level data have been limited, prompting efforts to better characterise its epidemiology and treatment.
The study identified 222 newly diagnosed cases using national registry data. Incidence rose from 0.7 per 100,000 person-years in 1999–2001 to 4.5 in 2014–2016, while prevalence increased from 2.2 to 8.8 per 100,000 individuals between 1999–2001 and 2017–2019.
These findings indicate a marked increase in the recognised burden of paediatric autoimmune hepatitis over two decades.
Most Patients Require Long-Term Immunosuppression
Treatment patterns aligned with standard immunosuppressive strategies. Within the first year following diagnosis, 95% of patients received prednisolone, while 72% were treated with thiopurines and 10% with tacrolimus.
Five years after diagnosis nearly all patients had received prednisolone, with thiopurine use rising to 80% and tacrolimus to 20%. This escalation reflects the need for sustained disease control in a proportion of patients.
High Native Liver Survival Despite Aggressive Disease
Clinical outcomes were encouraging. Native liver survival remained high, at 97% five years after diagnosis and 93% at ten years, indicating that most patients avoided liver transplantation during follow-up.
Registry-Based Approach Supports Robust Case Identification
The analysis used a nationwide, register-based design, with case identification supported by an algorithm demonstrating high sensitivity and perfect specificity. This approach enabled longitudinal assessment of incidence, prevalence, medication exposure, and transplantation outcomes across a large population.
Registry data depend on correct coding of diagnoses and procedures. Some cases may be misclassified, and laboratory, biopsy, or dosing details are not captured, so trends reflect population patterns rather than individual patient nuance.
Implications for Clinical Awareness and Management
The increasing incidence and prevalence of paediatric autoimmune hepatitis highlight the importance of clinical awareness and early diagnosis. At the same time, high native liver survival suggests that current immunosuppressive regimens, including corticosteroids and thiopurines, are effective in maintaining long-term liver function in most patients.
Reference
Malham M et al. Incidence, Prevalence, Medication Use, and Transplant Rates in Paediatric Autoimmune Hepatitis-A Nationwide Cohort Study. Liver Int. 2026;DOI:10.1111/liv.70625.
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