Overlap Syndrome Between Autoimmune Hepatitis and Primary Sclerosing Cholangitis in a Seven-Year-Old Child: A Case Report
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.
Fields of Study & Subjects
Liver Diseases and Immunity
Gallbladder and Bile Duct Disorders
Pediatric Hepatobiliary Diseases and Treatments
Publication details
- Journal / source
- Cureus
- Publisher
- Springer Science and Business Media LLC
- Volume
- 18
- Issue
- 5
- Pages
- e109381–e109381
- Publication date
- 21 May 2026
- Language
- EN