Pembrolizumab-Induced Vanishing Bile Duct Syndrome: a Case Report

Immune check-point inhibitors (ICPI) are becoming frequently used as first- or second-line therapy in different types of cancer. ICPI-related hepatotoxicity is usually immune mediated and can vary in severity from mild hepatitis to acute liver failure. We report the case of a patient with non-small cell lung cancer who developed severe hepatotoxicity following the first administration of pembrolizumab. Unexpectedly, pathological findings revealed a non-immune-mediated cholestatic liver injury consistent with vanishing bile duct syndrome (VBDS). VBDS is a rare disease characterized by progressive destruction of intrahepatic bile ducts, which has been associated with different conditions, including adverse drug reactions. Treatment consists in discontinuation of the offending drug while steroid-based treatment is not recommended.