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a hepatologie

Gastroenterology and Hepatology

Gastroent Hepatol 2022; 76(6): 499–503. doi: 10.48095/ccgh2022499.

“Vanishing bile duct syndrome” as a manifestation of drug-induced liver damage in a patient after polytrauma

Lucia Horniaková1, Miroslav Žigrai2, Silvia Žigraiová2, Xénia Faktorová2, Světlana Adamcová-Selčanová3, Roman Belica2, D. Guga2, Milan Vyskočil  2

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Vanishing bile duct syndrome is a type of drug-induced cholestatic liver injury that occurs when the body is exposed to drugs or other foreign substances. We hospitalized a 44-year-old, so far healthy, male with gradually developing painless icterus and pruritus of the whole body after severe polytrauma. The patient had previously undergone a complex treatment (antibio­tics, antifungals, parenteral nutrition, LMWH). Laboratory tests revealed a several-fold elevation of total and conjugated bilirubin with a 3-fold increase in ALP levels, no significant elevation of transaminases, a decrease in prothrombin time and IgG4 antibody positivity. An MRCP scan was performed revealing irregular intrahepatic bile ducts, a dilated common bile duct and characteristics of primary or secondary sclerosing cholangitis. Further examinations ruled out infectious, metabolic, toxic and oncological causes of hepatopathy. A liver bio­psy ruled out both autoimmune hepatitis and IgG4 hepatopathy and displayed images of “vanishing bile duct syndrome” in drug-induced liver injury (DILI). Empirical treatment with high-dose intravenous N-acetylcysteine and other hepatoprotective agents led to a gradual decrease of bilirubin, normalization of coagulation parameters and remission of icterus and clinical complaints. This case report points to a cholestatic type of drug-induced liver injury with histologically confirmed vanishing bile duct syndrome and the need to think of DILI as one of the causes of the otherwise unexplained hepatopathy.


cholangitis, DILI, liver diseases, IgG4, vanishing bile duct syndrome

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