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Vanishing Bile Duct Syndrome Associated With Estrogen
John Benfield1, Raj A Shah2, Douglas J Grider3,4
1Department of Internal Medicine, Virginia Tech Carilion School of Medicine, Roanoke, VA.
ACG Case Reports Journal
|August 3, 2022
Summary
Vanishing bile duct syndrome (VBDS) is a rare liver condition causing bile duct loss. This case highlights a potential link between hormonal therapies and VBDS development, necessitating further investigation.
Area of Science:
- Hepatology
- Endocrinology
- Pathology
Background:
- Vanishing bile duct syndrome (VBDS) is a cholestatic liver disease with diverse causes and unclear pathophysiology.
- It is characterized by the loss of intrahepatic bile ducts (biliary ductopenia) and persistent cholestasis.
Observation:
- An 18-year-old woman presented with cholestatic liver injury and intrahepatic biliary ductopenia.
- Her condition followed treatment with oral contraceptives and intravenous estrogen for uterine bleeding.
Findings:
- The patient showed no significant improvement in liver biomarkers a year after initial treatment.
- Intrahepatic biliary ductopenia persisted, indicating a severe form of VBDS.
Implications:
- This case suggests a potential association between specific hormonal therapies and the development of VBDS.
- The unclear pathophysiology of VBDS underscores the need for further research into its triggers and mechanisms.
- The patient's referral for liver transplantation highlights the severity and limited treatment options for advanced VBDS.
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