An Elusive Diagnosis of Vanishing Bile Duct Syndrome in an HIV Patient

Vanishing bile duct syndrome (VBDS) is an extremely rare disorder characterized by the progressive destruction of intrahepatic bile ducts, leading to cholestasis and liver dysfunction. It is most commonly associated with autoimmune and infectious conditions but can also occur in immunocompromised states such as human immunodeficiency virus (HIV). We report the case of a 58-year-old Hispanic woman with HIV who presented with cyclical fevers, transaminitis, hyperbilirubinemia, and hyponatremia over a 3-month period. Laboratory workup revealed marked cholestasis with severe elevations in alkaline phosphatase. A liver biopsy confirmed VBDS with a significant reduction in intrahepatic bile ducts. The patient continued antiretroviral treatment, prophylactic antibiotics, and was started on ursodiol, resulting in improvement in liver tests. An extensive workup was done to investigate for an underlying opportunistic infection or lymphoproliferative disease as a culprit for VBDS. VBDS, while exceedingly rare in HIV-positive patients, is likely immune mediated, involving T-cell dysfunction and cytokine-mediated biliary epithelial cell apoptosis. While drug-induced hepatotoxicity from antiretroviral therapy is a possible contributing factor, it is crucial to consider other underlying etiologies in immunocompromised patients such as infections and lymphoproliferative disorders. VBDS should be considered in HIV-positive patients with unexplained liver dysfunction, particularly those with low CD4 counts. Early recognition and diagnosis are crucial for initiating appropriate treatment and preventing further liver damage. This case highlights the diagnostic complexity and need for ongoing research into the interplay between immunosuppression, cholangiopathy, and liver dysfunction in HIV patients.