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

作　　者：Tingting Lv Haitian Yu Xiao Han Aileen Wee Jimin Liu Min Li Jinghang Xu Xiaoli Hu Jia Li Weijia Duan Tailing Wang Jidong Jia Xinyan Zhao 

机构地区：[1]Liver Research Center,Beijing Friendship Hospital,Capital Medical University,National Clinical Research Center for Digestive Diseases,Beijing,China [2]Department of Pathology,Yong Loo Lin School of Medicine,National University of Singapore,National University Hospital,Singapore [3]Department of Pathology and Molecular Medicine,Faculty of Health Sciences,McMaster University,Hamilton,Ontario,Canada [4]Clinical Epidemiology and Evidence-Based Medicine Unit,National Clinical Research Center for Digestive Diseases,Beijing Friendship Hospital,Capital Medical University,Beijing,China [5]Department of infectious disease,Peking University First Hospital,Beijing,China [6]Department of infectious disease,Heilongjiang Province Hospital,Heilongjiang,China [7]Department of Hepatology,Tianjin Second People’s Hospital,Tianjin,China [8]Department of Pathology,China-Japan Friendship Hospital,Beijing,China

出　　处：《Journal of Clinical and Translational Hepatology》2023年第5期1161-1169,共9页临床与转化肝病杂志（英文版）

基　　金：supported by National Natural Science Foundation of China(No.82100633 to LTT).

摘　　要：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.

关 键 词：Bile duct loss PROGNOSIS CHOLESTASIS Foam cells Advanced fibrosis 

分 类 号：R575.7[医药卫生—消化系统]