Overlap Syndrome Between Autoimmune Hepatitis and Primary Sclerosing Cholangitis in a Seven-Year-Old Child: A Case Report

Overlap syndrome between autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) is a rare condition in children and represents a diagnostic challenge due to the lack of standardized diagnostic criteria and the frequent presence of atypical clinical manifestations. We report the case of a seven-year-old girl presenting with chronic jaundice, severe hepatocellular cytolysis, polyclonal hypergammaglobulinemia, and biliary abnormalities detected on MRI. Liver histology confirmed AIH. The diagnosis of AIH-PSC overlap syndrome was established, and treatment with prednisone and azathioprine was initiated, resulting in significant clinical and biochemical improvement. This case highlights the importance of early diagnostic suspicion and multidisciplinary management in children presenting with complex autoimmune liver disease.

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Publication Details

Journal
Cureus
Published
2026-05-21
DOI
https://doi.org/10.7759/cureus.109381
Primary Topic
Liver Diseases and Immunity
Type
article
Field-Weighted Citation Impact
0.00
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Overlap Syndrome Between Autoimmune Hepatitis and Primary Sclerosing Cholangitis in a Seven-Year-Old Child: A Case Report

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Overlap Syndrome Between Autoimmune Hepatitis and Primary Sclerosing Cholangitis in a Seven-Year-Old Child: A Case Report

Imane Kamaoui, Abdeladim Babakhouya, Maria Rkain, Amal Hamamı, Chaymae Cherrabi
article en

Abstract

Overlap syndrome between autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) is a rare condition in children and represents a diagnostic challenge due to the lack of standardized diagnostic criteria and the frequent presence of atypical clinical manifestations. We report the case of a seven-year-old girl presenting with chronic jaundice, severe hepatocellular cytolysis, polyclonal hypergammaglobulinemia, and biliary abnormalities detected on MRI. Liver histology confirmed AIH. The diagnosis of AIH-PSC overlap syndrome was established, and treatment with prednisone and azathioprine was initiated, resulting in significant clinical and biochemical improvement. This case highlights the importance of early diagnostic suspicion and multidisciplinary management in children presenting with complex autoimmune liver disease.

Cureus
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Liver Diseases and Immunity
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