Introduction: Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disorder characterized by severe pruritus and elevated bile acids, carrying risks for both maternal well-being and fetal outcomes. Management can be particularly challenging in patients with underlying cholestatic liver diseases, even after liver transplantation.
Introduction:
Intrahepatic cholestasis of pregnancy (ICP) is a pregnancy-specific liver disorder characterized by severe pruritus and elevated bile acids, carrying risks for both maternal well-being and fetal outcomes. Management can be particularly challenging in patients with underlying cholestatic liver diseases, even after liver transplantation. Therapeutic options for refractory ICP remain limited when conventional treatment fails to adequately control symptoms and bile acid levels.
Problem Statement:
Women with a history of cholestatic disorders such as Alagille syndrome may remain susceptible to severe cholestatic complications during pregnancy despite successful liver transplantation. Refractory ICP can lead to debilitating pruritus and increased obstetric risk, highlighting the need for effective alternative therapies when standard treatments prove insufficient.
Summary:
This report describes the use of an ileal bile acid transporter (IBAT) inhibitor in a liver-transplanted woman with Alagille syndrome who developed refractory intrahepatic cholestasis of pregnancy. The case highlights the persistent vulnerability to cholestatic dysfunction that may remain despite transplantation, particularly under the physiological stress of pregnancy. Conventional therapeutic measures were insufficient to adequately control the patient's cholestatic symptoms, prompting consideration of an IBAT inhibitor. By reducing enterohepatic bile acid recirculation, IBAT inhibition offers a mechanistically targeted approach to lowering systemic bile acid burden and alleviating cholestatic symptoms. The reported experience suggests that this strategy may provide meaningful symptomatic and biochemical improvement in highly selected patients with difficult-to-manage ICP. Beyond its immediate clinical relevance, the case raises important questions regarding the potential role of IBAT inhibitors in pregnancy-associated cholestatic disorders, particularly among women with underlying genetic cholestatic diseases or prior liver transplantation. Although conclusions are necessarily limited by the single-patient nature of the report, the findings highlight a promising therapeutic avenue in an area where treatment options remain scarce. This case contributes to the emerging evidence supporting bile acid–targeted therapies and may stimulate future studies evaluating the safety and efficacy of IBAT inhibitors in severe or refractory ICP.
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