Unveiling the Cholestatic Phenotype: A Case Report of Autoimmune Hepatitis with Severe Hyperbilirubinemia

Background Autoimmune hepatitis (AIH) is typically characterized by chronic liver inflammation, but in rare cases, it can present with a cholestatic phenotype, marked by severe hyperbilirubinemia and elevated liver enzymes. This atypical form poses diagnostic challenges, often mimicking other cholestatic liver diseases, requiring thorough evaluation, including liver biopsy, for accurate diagnosis. Case Presentation We report a 52-year-old Asian woman with cholestasis, including jaundice and fatigue, following a febrile illness. Despite initial negative results for typical AIH markers, a liver biopsy confirmed AIH, showing interface hepatitis and cholestasis. Conclusion Treatment with azathioprine and prednisolone led to rapid normalization of bilirubin levels. This case underscores the importance of considering AIH in cholestatic presentations and highlights the efficacy of timely steroid therapy in such cases.