Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value

Introduction and Objectives: Primary biliary cholangitis (PBC) may progress to clinically significant portal hypertension (CSPH) before the development of cirrhosis. This study aimed to investigate CSPH incidence as well as the clinicopathological characteristics and predictive value of these featur...

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Main Authors: Ying Zhang, Huaying Lai, Jing Chen, Ruimin Lai, Xiaoyu Lin, Shan Lin, Bingping Liu, Qiuxiang Lin, Bin Wang, Qi Zheng
Format: Article
Language:English
Published: Elsevier 2025-01-01
Series:Annals of Hepatology
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Online Access:http://www.sciencedirect.com/science/article/pii/S1665268124003600
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author Ying Zhang
Huaying Lai
Jing Chen
Ruimin Lai
Xiaoyu Lin
Shan Lin
Bingping Liu
Qiuxiang Lin
Bin Wang
Qi Zheng
author_facet Ying Zhang
Huaying Lai
Jing Chen
Ruimin Lai
Xiaoyu Lin
Shan Lin
Bingping Liu
Qiuxiang Lin
Bin Wang
Qi Zheng
author_sort Ying Zhang
collection DOAJ
description Introduction and Objectives: Primary biliary cholangitis (PBC) may progress to clinically significant portal hypertension (CSPH) before the development of cirrhosis. This study aimed to investigate CSPH incidence as well as the clinicopathological characteristics and predictive value of these features for the prognosis of patients with PBC, especially at early histologic stage. Patients and Methods: Patients diagnosed with PBC between January 2013 and April 2022 were retrospectively enrolled. The prognostic value of baseline clinicopathological characteristics for long-term outcomes in PBC patients with CSPH was assessed using Kaplan–Meier survival analysis and COX regression analysis. Results: Among 280 patients with PBC, 104 underwent liver biopsy and 68 were at early histologic stage. CSPH was present in 47.2 % of participants with 20.6 % at early histologic stage. CSPH was a risk factor for predicting the liver transplant-free survival in PBC patients (hazard ratio [HR], 6.78; 95 % CI, 2.94–15.63), especially those at early stage. Perisinusoidal fibrosis and nodular regenerative hyperplasia (NRH) were common histopathological features in PBC patients with CSPH at the early stages. Fibrous septa formation in the hepatic lobules (HR, 4.85; 95 % CI, 1.51–15.52) and cholestasis (HR, 7.70; 95 % CI, 2.56–23.18) were independent predictors of adverse outcomes. Conclusions: CSPH indicates an increased risk of adverse outcomes in PBC patients, especially those in early histologic stage. Perisinusoidal fibrosis and NRH are valuable histological features of CSPH in patients with early-stage PBC. Identification of clinicopathological features and assessment of portal hypertension (especially at early stage), contribute to the development of personalized strategies.
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spelling doaj-art-e85fd8f4bea342dea7b5fc815fb838662025-08-20T02:01:51ZengElsevierAnnals of Hepatology1665-26812025-01-0130110157710.1016/j.aohep.2024.101577Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic valueYing Zhang0Huaying Lai1Jing Chen2Ruimin Lai3Xiaoyu Lin4Shan Lin5Bingping Liu6Qiuxiang Lin7Bin Wang8Qi Zheng9Department of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR ChinaDepartment of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR ChinaDepartment of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR ChinaDepartment of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR ChinaDepartment of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR ChinaDepartment of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR ChinaDepartment of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR ChinaDepartmentofHepatology, Mengchao Hepatobiliary hospital of Fujian Medical University, Fuzhou 350025, Fujian Province, PR ChinaDepartment of Pathology and Institute of Oncology, The School of Basic Medical Sciences, Fujian Medical University, Fuzhou, Fujian Province, PR China; Diagnostic Pathology Centre, Fujian Medical University, Fuzhou, Fujian Province, PR China; Department of Pathology, Mengchao Hepatobiliary Hospital of Fujian Medical University, Fuzhou, 350025, Fujian Province, PR China; Corresponding authors.Department of Hepatology, Hepatology Research Institute, The First Affiliated Hospital, Fujian Medical University, Fuzhou 350005, Fujian Province, PR China; Department of Hepatology, National Regional Medical Center, Binhai Campus of the First Affiliated Hospital, Fujian Medical University, Fuzhou 350212, Fujian Province, PR China; Corresponding authors.Introduction and Objectives: Primary biliary cholangitis (PBC) may progress to clinically significant portal hypertension (CSPH) before the development of cirrhosis. This study aimed to investigate CSPH incidence as well as the clinicopathological characteristics and predictive value of these features for the prognosis of patients with PBC, especially at early histologic stage. Patients and Methods: Patients diagnosed with PBC between January 2013 and April 2022 were retrospectively enrolled. The prognostic value of baseline clinicopathological characteristics for long-term outcomes in PBC patients with CSPH was assessed using Kaplan–Meier survival analysis and COX regression analysis. Results: Among 280 patients with PBC, 104 underwent liver biopsy and 68 were at early histologic stage. CSPH was present in 47.2 % of participants with 20.6 % at early histologic stage. CSPH was a risk factor for predicting the liver transplant-free survival in PBC patients (hazard ratio [HR], 6.78; 95 % CI, 2.94–15.63), especially those at early stage. Perisinusoidal fibrosis and nodular regenerative hyperplasia (NRH) were common histopathological features in PBC patients with CSPH at the early stages. Fibrous septa formation in the hepatic lobules (HR, 4.85; 95 % CI, 1.51–15.52) and cholestasis (HR, 7.70; 95 % CI, 2.56–23.18) were independent predictors of adverse outcomes. Conclusions: CSPH indicates an increased risk of adverse outcomes in PBC patients, especially those in early histologic stage. Perisinusoidal fibrosis and NRH are valuable histological features of CSPH in patients with early-stage PBC. Identification of clinicopathological features and assessment of portal hypertension (especially at early stage), contribute to the development of personalized strategies.http://www.sciencedirect.com/science/article/pii/S1665268124003600Early histologyHistopathological featurePortal hypertensionPrimary biliary cholangitisPrognosis
spellingShingle Ying Zhang
Huaying Lai
Jing Chen
Ruimin Lai
Xiaoyu Lin
Shan Lin
Bingping Liu
Qiuxiang Lin
Bin Wang
Qi Zheng
Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value
Annals of Hepatology
Early histology
Histopathological feature
Portal hypertension
Primary biliary cholangitis
Prognosis
title Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value
title_full Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value
title_fullStr Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value
title_full_unstemmed Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value
title_short Clinically significant portal hypertension in patients with primary biliary cholangitis: Clinicopathological features and prognostic value
title_sort clinically significant portal hypertension in patients with primary biliary cholangitis clinicopathological features and prognostic value
topic Early histology
Histopathological feature
Portal hypertension
Primary biliary cholangitis
Prognosis
url http://www.sciencedirect.com/science/article/pii/S1665268124003600
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