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. 2023 Oct 28;11(5):1161-1169.
doi: 10.14218/JCTH.2022.00039. Epub 2023 Apr 4.

Histopathological Features Predicting Long-term Clinical Outcomes in Patients with Vanishing Bile Duct Syndrome

Tingting Lv  1 , Haitian Yu  1 , Xiao Han  1 , Aileen Wee  2 , Jimin Liu  3 , Min Li  4 , Jinghang Xu  5 , Xiaoli Hu  6 , Jia Li  7 , Weijia Duan  1 , Tailing Wang  8 , Jidong Jia  1 , Xinyan Zhao  1
Affiliations

Histopathological Features Predicting Long-term Clinical Outcomes in Patients with Vanishing Bile Duct Syndrome

Tingting Lv et al. J Clin Transl Hepatol. .

Abstract

Background and aims: The clinicopathological features and long-term outcomes of patients with vanishing bile duct syndrome (VBDS) have yet to be elucidated. The study aims to investigate these features and identify factors associated with poor prognosis.

Methods: This multicenter retrospective study recruited patients with liver biopsy-proven VBDS who were followed up at five hospitals in northern China from January 2003 to April 2022. Clinical and pathological data at time of biopsy were reviewed. Clinical outcomes including cirrhosis, decompensation events, liver transplantation (LT), and liver-related death were recorded. Cox regression analysis was used to identify the risk factors associated with poor outcomes.

Results: A total of 183 patients were included. The median age was 47 years, with 77.6% being women. During a median follow-up of 4.8 years, 88 patients developed compensated or decompensated cirrhosis, 27 died, and 15 received LT. Multivariate Cox regression analysis showed that hepatocellular cholestasis (HR 2.953, 95% CI: 1.437-6.069), foam cells (HR 2.349, 95% CI: 1.092-5.053), and advanced fibrosis (HR 2.524, 95% CI: 1.313-4.851) were independent predictors of LT or liver-related deaths. A nomogram formulated with the above factors showed good consistency with a concordance index of 0.746 (95% CI: 0.706-0.785).

Conclusions: Nearly half of VBDS patients studied progressed to end-stage liver disease and 23% of them had LT or liver-related death within two years of diagnosis. Hepatocellular cholestasis, foam cells and advanced fibrosis rather than the degree of bile duct loss or underlying etiologies were independently associated with poor prognosis in VBDS patients.

Keywords: Advanced fibrosis; Bile duct loss; Cholestasis; Foam cells; Prognosis.

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Conflict of interest statement

JJ has been an executive associate editor of Journal of Clinical and Translational Hepatology since 2013. The other authors have no conflict of interests related to this publication.

Figures

Fig. 1
Fig. 1. Flowchart of the retrospective multicenter cohort of patients diagnosed with VBDS on liver biopsy.
A final total of 183 patients were included, of which 42 succumbed to liver-related deaths or required liver transplantation. VBDS, vanishing bile duct syndrome.
Fig. 2
Fig. 2. Kaplan-Meier plots of transplant-free survival in VBDS patients.
Stratification by (A) total bilirubin (TBIL) levels, (B) hepatocellular cholestasis, (C) foam cells and (D) fibrosis stage. Significant differences were observed in all the parameters analyzed (p<0.05). VBDS, vanishing bile duct syndrome.
Fig. 3
Fig. 3. Prominent histopathological findings in the liver biopsies of VBDS patients with clinical outcomes of liver transplantation or death.
(A–C) VBDS accompanied by cholestasis but without obvious inflammation or significant fibrosis. (D–F) VBDS accompanied by clusters of foam cells but without obvious inflammation or fibrosis. CK7+ intermediate hepatocytes are prominent. (A, D: hematoxylin and eosin; B, E: Masson trichrome; C, F: CK7). VBDS, vanishing bile duct syndrome.
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