Korean J Gastroenterol.  2024 Dec;84(6):288-292. 10.4166/kjg.2024.133.

Idiopathic Vanishing Bile Duct Syndrome in a Young Female: A Case Report

• Kim MJ
• Cho HM
• Hong YM
• Yoon KT

• KMID: 2563346
• DOI: http://doi.org/10.4166/kjg.2024.133

Abstract

Vanishing bile duct syndrome (VBDS) is characterized by the progressive loss and destruction of the intrahepatic bile ducts, leading to bile stasis and associated symptoms such as jaundice. This condition is commonly associated with drug side effects, infections, neoplasms, and autoimmune diseases, but the precise mechanism of its development is unclear. Although VBDS can be diagnosed based on the patient's symptoms and disease progression, a liver biopsy is essential for confirmation, and the prognosis can vary significantly. This paper presents a rare case of a young female patient diagnosed with idiopathic VBDS after undergoing a liver biopsy to investigate unexplained jaundice. The patient's liver function improved partially after an ursodeoxycholic acid and prednisolone treatment.

Keyword

Jaundice; Cholestasis; Bile ducts

Figure

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  Fig. 1 Patient's computed tomography images. The liver and bile ducts showed no abnormalities. (A) Axial image, (B) Coronal image.

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  Fig. 2 Pathologic findings of a liver biopsy. The hepatocytes exhibited a striking reaction with the K7 antibody through the biopsy specimen. The red arrow indicates an interlobular bile duct rarely seen in this sample, while the blue arrow indicates a portal area devoid of bile duct. (A) Hematoxylin and Eosin stain, ×200, (B) K7 stain, ×200, (C) K7 stain, ×40.

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  Fig. 3 Changes in (A) AST, ALT, ALP, GGT (IU/L), (B) total bilirubin (mg/dL), and INR levels for 186 days. The liver biopsy was performed on the sixth day of hospitalization, and prednisolone administration was initiated on the 13th day of hospitalization. AST, aspartate aminotransferase; ALT, alanine aminotransferase; ALP, alkaline phosphatase; GGT, gamma-glutamyl transferase.

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