Pediatr Gastroenterol Hepatol Nutr.  2021 Mar;24(2):154-163. 10.5223/pghn.2021.24.2.154.

Autoimmune Sclerosing Cholangitis in Children: A Prospective Case-Control Study

• Kumar N
• Poddar U
• Yadav R
• Lal H
• Pani K
• Yachha SK
• Srivastava A
• Pandey R

• KMID: 2513659
• DOI: http://doi.org/10.5223/pghn.2021.24.2.154

Abstract

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.

Keyword

Autoimmune sclerosing cholangitis; Autoimmune liver disease; Autoimmune hepatitis; Magnetic resonance cholangiography