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

Czech version

Authors: X. Faktorová ¹; M. Žigrai ¹; Žigraiová S. ¹; Horniaková L. ²; S. Adamcová Selčanová ³; Belica R. ¹; Guga D. ¹; Vyskočil M. ¹
Authors‘ workplace: Interná klinika SZU, Univerzitná nemocnica – Nemocnica svätého Michala, a. s., Bratislava ¹; III. interná klinika LF UK a UN Bratislava ²; Hepatologicko-gastroenterologické a transplantačné oddelenie (HEGITO), II. interná klinika SZU, Banská Bystrica ³
Published in: Gastroent Hepatol 2022; 76(6): 499-503
Category: Hepatology: case report
doi: https://doi.org/10.48095/ccgh2022499

Overview

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.

Keywords:

cholangitis – IgG4 – hepatopathy – DILI – vanishing bile duct syndrome

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