Drug-Induced Vanishing Bile Duct Syndrome: From Pathogenesis to Diagnosis and Therapeutics

2021 ◽  
Author(s):  
Fernando Bessone ◽  
Nidia Hernández ◽  
Mario Tanno ◽  
Marcelo G. Roma
Keyword(s):  
Bile Duct ◽  
Diagnostic Tools ◽  
Drug Induced ◽  
Vanishing Bile Duct Syndrome ◽  
Beneficial Effects ◽  
Intrahepatic Bile Ducts ◽  
Chronic Cholestasis ◽  
Vanishing Bile Duct ◽  
Therapeutic Alternatives ◽  
Duct Syndrome

AbstractThe most concerned issue in the context of drug/herb-induced chronic cholestasis is vanishing bile duct syndrome. The progressive destruction of intrahepatic bile ducts leading to ductopenia is usually not dose dependent, and has a delayed onset that should be suspected when abnormal serum cholestasis enzyme levels persist despite drug withdrawal. Immune-mediated cholangiocyte injury, direct cholangiocyte damage by drugs or their metabolites once in bile, and sustained exposure to toxic bile salts when biliary epithelium protective defenses are impaired are the main mechanisms of cholangiolar damage. Current therapeutic alternatives are scarce and have not shown consistent beneficial effects so far. This review will summarize the current literature on the main diagnostic tools of ductopenia and its histological features, and the differential diagnostic with other ductopenic diseases. In addition, pathomechanisms will be addressed, as well as the connection between them and the supportive and curative strategies for ductopenia management.

scholarly journals Stevens–Johnson syndrome and acute vanishing bile duct syndrome after the use of amoxicillin and naproxen in a child

2019 ◽  
Vol 47 (9) ◽  
pp. 4537-4543 ◽  
Author(s):  
Lu Li ◽  
Sujun Zheng ◽  
Yu Chen
Keyword(s):  
Case Report ◽  
Bile Duct ◽  
Epithelial Cells ◽  
Hypersensitivity Reaction ◽  
Stevens Johnson Syndrome ◽  
Drug Induced ◽  
Vanishing Bile Duct Syndrome ◽  
Johnson Syndrome ◽  
Vanishing Bile Duct ◽  
Duct Syndrome

We present the case report of a 6-year-old patient who developed Stevens–Johnson syndrome (SJS) and acute vanishing bile duct syndrome (VBDS) after taking oral amoxicillin and naproxen. SJS, an immune complex-mediated hypersensitivity reaction involving the skin and mucosa, is usually drug-induced, and it can lead to systemic symptoms. Acute VBDS is rare, often presenting with progressive loss of the intrahepatic biliary tract. VBDS is an immune-mediated bile duct-associated disease, and immunological damage to the bile duct system is an important mechanism for VBDS. Serious drug-induced liver injury (DILI) is also associated with immunity. The drug acts as a hapten with keratin on the surface of biliary epithelial cells. The autoantibodies produced by this action can damage the bile duct epithelial cells and cause the bile duct to disappear. SJS is a serious type of polymorphic erythema that is mainly considered to be a hypersensitivity reaction to drugs, and it may involve multiple factors.  The patient in this case report was treated with glucocorticoids, plasma exchange, ursodeoxycholic acid, and traditional Chinese medicine. He recovered completely within 5 months. This case report indicates that caution should be used because amoxicillin and naproxen can cause SJS and VBDS in children.

scholarly journals Successful liver transplantation for drug-induced vanishing bile duct syndrome

2020 ◽  
Vol 13 (1) ◽  
pp. e233052 ◽  
Author(s):  
Ahmed Hashim ◽  
Ashley Barnabas ◽  
Rosa Miquel ◽  
Kosh Agarwal
Keyword(s):  
Liver Transplantation ◽  
Bile Duct ◽  
Graft Function ◽  
Drug Induced ◽  
Vanishing Bile Duct Syndrome ◽  
Clinical Presentations ◽  
Number Of Patients ◽  
Wide Range ◽  
Vanishing Bile Duct ◽  
Duct Syndrome

Drug-induced cholestasis has a wide range of clinical presentations, and in a small number of patients, it can progress to severe ductopenia. A 63-year-old woman was referred to our department with progressive cholestasis and hyperbilirubinaemia following a course of flucloxacillin. Despite the comprehensive laboratory, imaging and genetic investigations, no other cause for the cholestasis was demonstrated. Sequential liver biopsies confirmed the development of vanishing bile duct syndrome. She developed significant cachexia and pruritus that did not respond to medical therapy, and hence she was listed for liver transplantation. She underwent liver transplantation 6 months after the initial drug-induced injury. She has remained well with good graft function at 1-year follow-up. The case highlights an extreme form of drug-induced ductopenia and underscores the need for meticulous hepatology input and consideration of liver transplantation in some patients.

scholarly journals Hepatic Ductopenia and Vanishing Bile Duct Syndrome Following Anabolic Androgenic Steroid Use: A Case Report and Literature Review.

2018 ◽  
Vol 12 (4) ◽  
Author(s):  
Adam Edward Mazzetti ◽  
Resheed Alkhiari ◽  
Vidhya Nair ◽  
Ted Xenodemetropoulos
Keyword(s):  
Bile Duct ◽  
Anabolic Androgenic Steroid ◽  
Vanishing Bile Duct Syndrome ◽  
Androgenic Steroid ◽  
Intrahepatic Bile Ducts ◽  
Genetic Abnormalities ◽  
Primary Neoplasms ◽  
Vanishing Bile Duct ◽  
Anomalies Génétiques ◽  
Duct Syndrome

Vanishing bile duct syndrome (VBDS) is a rare acquired disorder associated with progressive destruction and disappearance of intrahepatic bile ducts which eventually leads to cholestasis. VBDS has been linked to a variety of etiologies, including autoimmune disorders, infectious diseases, primary neoplasms, genetic abnormalities, and many classes of medications, including antibiotics, nonsteroidal anti-inflamatories (NSAIDs), anticonvulsants, antipsychotics and others. We present the case of VBDS associated with anabolic androgenic steroid (AAS) exposure in an otherwise healthy 29-year-old male. Resume Le syndrome de disparition des canaux biliaires (SDCB) est une affection acquise rare caractérisée par la destruction progressive et la disparition des canaux biliaires intrahépatiques entrainant une cholestase. Le SDCB a été associé à diverses étiologies, dont des affections auto-immunes, des maladies infectieuses, des néoplasmes primaires, des anomalies génétiques et de nombreuses classes de médicaments, incluant des antibiotiques, des anti-inflammatoires non stéroïdiens, des anticonvulsivants, des antipsychotiques et autres. Nous présentons un cas de SDCB associé à la prise d’un stéroïde androgénique anabolisant chez un homme de 29 ans par ailleurs en bonne santé.    

Drug Induced Vanishing Bile Duct Syndrome

2015 ◽  
Vol 110 ◽  
pp. S392-S393
Author(s):  
Lakshmi S. Yarlagadda ◽  
Olusesan Olotu ◽  
Belen Tesfaye ◽  
Andrew Sanderson ◽  
Charles Howell
Keyword(s):  
Bile Duct ◽  
Drug Induced ◽  
Vanishing Bile Duct Syndrome ◽  
Vanishing Bile Duct ◽  
Duct Syndrome
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