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Published on: May 29, 2020
Autoimmune hepatitis in children in Eastern Denmark
Joanna Vitfell-Pedersen1, Marianne Hørby Jørgensen, Klaus Müller
1Sarcoma Unit, Royal Marsden Hospital, London, UK
Insights
Autoimmune hepatitis in children shares similarities with adult cohorts but lacks female predominance. Early treatment with prednisolone and azathioprine may be more effective than prednisolone alone for pediatric autoimmune hepatitis.
Area of Science:
- Pediatric Hepatology
- Immunology
- Internal Medicine
Background:
- Autoimmune hepatitis (AIH) in childhood is a chronic, progressive inflammatory liver disease.
- Understanding the clinical and biochemical profiles of pediatric AIH is crucial for effective management.
- Previous studies on pediatric AIH cohorts provide a basis for comparison and further research.
Purpose of the Study:
- To compare the clinical and biochemical characteristics of 33 pediatric AIH patients with existing literature.
- To evaluate the impact of early treatment strategies on the disease course in children with AIH.
- To assess the effectiveness of different therapeutic regimens in achieving remission.
Main Methods:
- A prospective population-based cohort of pediatric patients diagnosed with AIH between January 1993 and September 2009.
- Retrospective data collection through examination of patient files.
- Analysis of clinical presentation, biochemical markers, liver biopsy findings, and treatment responses.
Main Results:
- The cohort comprised 33 children (16 girls, 17 boys) with predominantly type 1 AIH (29 patients).
- A significant proportion (69.7%) presented with symptoms mimicking acute viral hepatitis, with 69.6% already showing cirrhosis on liver biopsy.
- Combination therapy with prednisolone and azathioprine achieved remission in 60.6% of patients, compared to 24.2% treated with prednisolone alone; one patient did not achieve remission.
Conclusions:
- Pediatric AIH patients in this cohort exhibited characteristics similar to previously reported groups, with no observed female predominance.
- Early initiation of treatment combining prednisolone and azathioprine appears more effective than prednisolone monotherapy for pediatric AIH.
- Further randomized controlled trials are necessary to definitively compare these treatment regimens in pediatric populations.
Objectives:
Autoimmune hepatitis (AIH) in childhood is a progressive chronic inflammatory liver disease. The aim of this study was to compare the clinical and biochemical characteristics of 33 paediatric patients diagnosed as having AIH with earlier described cohorts, and to examine the effect of early treatment strategies on the course of disease.
Methods:
A population-based cohort of patients from January 1993 to September 2009 was identified prospectively, and the patient data were collected by a retrospective examination of the files.
Results:
Twenty-nine patients had type 1 AIH, 2 had type 2, and 2 could not be categorised. Among the 33 children, 16 (48.5%) were girls and 17 (51.5%) were boys. Twenty-three (69.7%) of the patients had symptoms at presentation indistinguishable from acute viral hepatitis, but in 16 (69.6%) of those the liver biopsy showed cirrhosis. Twenty (60.6%) patients were treated with prednisolone and azathioprine at the time of remission, whereas 8 (24.2%) were treated with prednisolone. One (3%) patient did not experience remission during the observation period.
Conclusions:
The patients in our study appeared similar to previously published cohorts, although a female predominance was not observed. Our data suggest that early treatment including both prednisolone and azathioprine could be more effective than prednisolone alone, even if randomised controlled paediatric studies comparing these 2 different treatment regimens are needed.
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