Vanishing Bile Duct Syndrome: A Differential Diagnosis for Painless Jaundice

Vanishing bile duct syndrome (VBDS) is a clinicopathological term coined to describe an acquired liver disease characterised by progressive destruction and disappearance of intrahepatic biliary ducts. We report the case of a 69-year-old female who presented with painless jaundice, fatigue, and weight loss. Initial blood workup revealed hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and a raised international normalised ratio. Para-aortic lymphadenopathy on computed tomography of the abdomen was biopsied to confirm the diagnosis of Hodgkin’s lymphoma. Vanishing bile duct syndrome is a paraneoplastic phenomenon of Hodgkin’s lymphoma, a recognised cause of cholestatic jaundice, and our patient’s liver biopsy was diagnostic of the same. Despite treatment with steroids, ursodeoxycholic acid, and chemotherapy, our patient passed away. This case report aims to highlight VBDS as a differential diagnosis for painless jaundice in the context of cholestatic liver dysfunction. We believe reporting such cases irrespective of their outcome will help raise awareness of VBDS among clinicians, thus bettering the rate at which it is diagnosed and treated, thereby improving patient outcomes.

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Publication Details

Journal
Cureus
Published
2024-09-05
DOI
https://doi.org/10.7759/cureus.68724
Citations
2
Primary Topic
Pediatric Hepatobiliary Diseases and Treatments
Type
article
Field-Weighted Citation Impact
1.15
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article

Vanishing Bile Duct Syndrome: A Differential Diagnosis for Painless Jaundice

Fatima Shahid, Gayatri Chakrabarty, Siona Sabu
2 citations
Cureus
Pediatric Hepatobiliary Diseases and Treatments
1.15
article

Vanishing Bile Duct Syndrome: A Differential Diagnosis for Painless Jaundice

Fatima Shahid, Gayatri Chakrabarty, Siona Sabu
article en
2 citations

Abstract

Vanishing bile duct syndrome (VBDS) is a clinicopathological term coined to describe an acquired liver disease characterised by progressive destruction and disappearance of intrahepatic biliary ducts. We report the case of a 69-year-old female who presented with painless jaundice, fatigue, and weight loss. Initial blood workup revealed hyperbilirubinemia, transaminitis, elevated alkaline phosphatase, and a raised international normalised ratio. Para-aortic lymphadenopathy on computed tomography of the abdomen was biopsied to confirm the diagnosis of Hodgkin’s lymphoma. Vanishing bile duct syndrome is a paraneoplastic phenomenon of Hodgkin’s lymphoma, a recognised cause of cholestatic jaundice, and our patient’s liver biopsy was diagnostic of the same. Despite treatment with steroids, ursodeoxycholic acid, and chemotherapy, our patient passed away. This case report aims to highlight VBDS as a differential diagnosis for painless jaundice in the context of cholestatic liver dysfunction. We believe reporting such cases irrespective of their outcome will help raise awareness of VBDS among clinicians, thus bettering the rate at which it is diagnosed and treated, thereby improving patient outcomes.

Cureus
Surrey and Sussex Healthcare NHS Trust (GB)
Gender equality
Openalex Percentile: Top 21%
Pediatric Hepatobiliary Diseases and Treatments
1.15
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Vanishing Bile Duct Syndrome: A Differential Diagnosis for Painless Jaundice — Fatima Shahid, Gayatri Chakrabarty, et al. · Cureus (2024) | TGRS Research Map | TGRS