Ileal Bile Acid Transporter Inhibitors for Symptomatic Cholestasis due to Vanishing Bile Duct Syndrome in Adults

Vanishing bile duct syndrome (VBDS) is a rare cholangiopathy that may follow drug-induced liver injury and can progress to liver failure. No established pharmacological treatment is currently available. We present a case of a 65-year-old man diagnosed with histologically confirmed and progressive symptomatic VBDS, probably sustained by drug-induced liver injury, who was successfully treated with a 4-week course of off-label ileal bile acid transporter inhibitor treatment of the management of pruritus. To our knowledge, this is among the first reports of successful ileal bile acid transporter inhibitor use in VBDS. This case highlights the potential role of bile acid modulation in managing severe symptomatic VBDS and underscores the importance of a proactive, multidisciplinary approach to optimize outcomes in complex cholangiopathies.