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Autoimmune Sclerosing Cholangitis in Children: A Prospective Case-Control Study
Nagendra Kumar1, Ujjal Poddar1, Rajnikant Yadav2
1Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India.
Insights
Autoimmune sclerosing cholangitis (ASC) affects 10.5% of children with autoimmune hepatitis (AIH). Magnetic resonance cholangiography (MRC) is recommended for select AIH patients with specific symptoms, not all.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Autoimmune Liver Diseases
Background:
- Autoimmune hepatitis (AIH) can overlap with primary sclerosing cholangitis, termed autoimmune sclerosing cholangitis (ASC).
- Previous studies using ERCP indicated a high prevalence of ASC in children.
- The utility of MRC for diagnosing ASC in pediatric AIH requires further investigation.
Purpose of the Study:
- To determine the prevalence of ASC in children with AIH using MRC.
- To compare the clinical presentation and outcomes of AIH and ASC in pediatric patients.
- To assess the prevalence of ASC in children with non-AIH cirrhosis.
Main Methods:
- Prospective study involving MRC in 38 children with AIH and 19 disease controls (Wilson disease).
- ASC diagnosis based on multiple biliary strictures and proximal dilatation identified via MRC.
- Detailed recording of clinical, laboratory, histopathological data, and treatment responses.
Main Results:
- Definite ASC changes were observed in 10.5% of children with AIH.
- No significant differences in clinical, laboratory, histological parameters, or treatment response between ASC and AIH groups.
- Abnormal MRC findings were noted in 36.8% of AIH cases and 42.1% of controls.
Conclusions:
- The prevalence of ASC in pediatric AIH is 10.5% when diagnosed by MRC.
- Suggests selective use of MRC for children with AIH presenting with cholestatic features, inflammatory bowel disease, or poor immunosuppression response.
- Highlights the importance of advanced imaging in diagnosing overlapping autoimmune liver conditions in children.
Purpose:
In children overlap of autoimmune hepatitis (AIH) and primary sclerosing cholangitis is labelled as autoimmune sclerosing cholangitis (ASC). The only prospective pediatric study showed a high prevalence of ASC by using endoscopic retrograde cholangiopancreatography. Aims of our study were to find the prevalence of ASC by using magnetic resonance cholangiography (MRC) in AIH and in non-AIH cirrhosis and to compare clinical presentation and outcome of AIH and ASC.
Methods:
Prospectively we did MRC in 38 children with AIH (cases) and 19 disease controls (Wilson disease). Multiple biliary strictures with proximal dilatation on MRC were taken as definitive changes of ASC. Detail clinical, laboratory parameters, liver histopathology and treatment outcome were recorded.
Results:
The median age of cases was 11.5 (3-18) years, 22 (57.9%) were girls and 28 (73.7%) were diagnosed as type 1 AIH. MRC was done in 11 children (28.9%) at the time of diagnosis and in 27 (71.1%) after a median follow-up of 2.5 (0.3-10) years. Abnormal MRC changes were seen in 14/38 (36.8%) of AIH and 8/19 (42.1%) of controls. However, definite changes of ASC were present in four (10.5%) children in AIH and none in controls. None of the clinical, laboratory, histological parameters and treatment response were significantly different between ASC and AIH groups.
Conclusion:
The prevalence of ASC in children with AIH was just 10.5%. We suggest MRC in select group with cholestatic features, inflammatory bowel disease and in those who showed poor response to immunosuppression instead of all children with AIH.
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