Case report demonstrates successful treatment of autoimmune hepatitis and primary sclerosing cholangitis overlap in a child, suggesting need for prompt diagnosis.
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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Cherrabi et al. (2026) studied this question.
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