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门脉纤维化与小胆管反应:在肝病进展中的病理生理作用及转化机遇

Portal Fibrosis and the Ductular Reaction: Pathophysiological Role in the Progression of Liver Disease and Translational Opportunities.

作者信息

Gupta Vikas, Sehrawat Tejasav S, Pinzani Massimo, Strazzabosco Mario

机构信息

Liver Center and Section of Digestive Diseases, Department of Internal Medicine, Yale University, New Haven, Connecticut.

UCL Institute for Liver & Digestive Health, Royal Free Hospital, London, United Kingdom; University of Pittsburgh Medical Center-Mediterranean Institute for Transplantation and Highly Specialized Therapies, Palermo, Italy.

出版信息

Gastroenterology. 2025 Apr;168(4):675-690. doi: 10.1053/j.gastro.2024.07.044. Epub 2024 Sep 7.


DOI:10.1053/j.gastro.2024.07.044
PMID:39251168
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11885590/
Abstract

A consistent feature of chronic liver diseases and the hallmark of pathologic repair is the so-called "ductular reaction." This is a histologic abnormality characterized by an expansion of dysmorphic cholangiocytes inside and around portal spaces infiltrated by inflammatory, mesenchymal, and vascular cells. The ductular reaction is a highly regulated response based on the reactivation of morphogenetic signaling mechanisms and a complex crosstalk among a multitude of cell types. The nature and mechanism of these exchanges determine the difference between healthy regenerative liver repair and pathologic repair. An orchestrated signaling among cell types directs mesenchymal cells to deposit a specific extracellular matrix with distinct physical and biochemical properties defined as portal fibrosis. Progression of fibrosis leads to vast architectural and vascular changes known as "liver cirrhosis." The signals regulating the ecology of this microenvironment are just beginning to be addressed. Contrary to the tumor microenvironment, immune modulation inside this "benign" microenvironment is scarcely known. One of the reasons for this is that both the ductular reaction and portal fibrosis have been primarily considered a manifestation of cholestatic liver disease, whereas this phenomenon is also present, albeit with distinctive features, in all chronic human liver diseases. Novel human-derived cellular models and progress in "omics" technologies are increasing our knowledge at a fast pace. Most importantly, this knowledge is on the edge of generating new diagnostic and therapeutic advances. Here, we will critically review the latest advances, in terms of mechanisms, pathophysiology, and treatment prospects. In addition, we will delineate future avenues of research, including innovative translational opportunities.

摘要

慢性肝病的一个一致特征以及病理修复的标志是所谓的“小胆管反应”。这是一种组织学异常,其特征是在被炎症、间充质和血管细胞浸润的门管区内部及周围,形态异常的胆管细胞增生。小胆管反应是一种高度受调控的反应,基于形态发生信号机制的重新激活以及多种细胞类型之间的复杂相互作用。这些相互作用的性质和机制决定了健康的再生性肝修复与病理性修复之间的差异。细胞类型之间精心编排的信号传导引导间充质细胞沉积具有特定物理和生化特性的细胞外基质,即门管区纤维化。纤维化的进展会导致广泛的结构和血管变化,即“肝硬化”。调节这种微环境生态的信号刚刚开始得到研究。与肿瘤微环境相反,对这种“良性”微环境内免疫调节的了解却很少。原因之一是,小胆管反应和门管区纤维化一直主要被认为是胆汁淤积性肝病的表现,而这种现象在所有慢性人类肝病中也存在,尽管具有独特的特征。新型人类来源的细胞模型和“组学”技术的进展正在迅速增加我们的知识。最重要的是,这些知识即将带来新的诊断和治疗进展。在此,我们将批判性地回顾在机制、病理生理学和治疗前景方面的最新进展。此外,我们将描绘未来的研究途径,包括创新的转化机会。

相似文献

[1]
Portal Fibrosis and the Ductular Reaction: Pathophysiological Role in the Progression of Liver Disease and Translational Opportunities.

Gastroenterology. 2025-4

[2]
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[3]
Nuclear Translocation of RELB Is Increased in Diseased Human Liver and Promotes Ductular Reaction and Biliary Fibrosis in Mice.

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[4]
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[5]
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[6]
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[7]
Comparison of murine steatohepatitis models identifies a dietary intervention with robust fibrosis, ductular reaction, and rapid progression to cirrhosis and cancer.

Am J Physiol Gastrointest Liver Physiol. 2019-10-21

[8]
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[9]
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[10]
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Am J Physiol Gastrointest Liver Physiol. 2017-8-1

引用本文的文献

[1]
Hepatic Stellate Cell TM4SF1 Accelerates Hepatic Fibrosis Progression Via Interacting With the Tyrosine Kinase c-Src.

Cell Mol Gastroenterol Hepatol. 2025-6-21

本文引用的文献

[1]
Prominent role of gut dysbiosis in the pathogenesis of cystic fibrosis-related liver disease in mice.

J Hepatol. 2024-9

[2]
A Phase 3 Trial of Seladelpar in Primary Biliary Cholangitis.

N Engl J Med. 2024-2-29

[3]
Modulation of canonical Wnt signaling regulates peribiliary mesenchymal identity during homeostasis and injury.

Hepatol Commun. 2024-2-1

[4]
Single-cell, single-nucleus, and spatial transcriptomics characterization of the immunological landscape in the healthy and PSC human liver.

J Hepatol. 2024-5

[5]
The Diagnostic Value of FibroTest and Hepascore as Non-Invasive Markers of Liver Fibrosis in Primary Sclerosing Cholangitis (PSC).

J Clin Med. 2023-12-7

[6]
Efficacy and Safety of Elafibranor in Primary Biliary Cholangitis.

N Engl J Med. 2024-2-29

[7]
Galectin-3: therapeutic targeting in liver disease.

Expert Opin Ther Targets. 2023

[8]
Hepatic farnesoid X receptor is necessary to facilitate ductular reaction and expression of heme biosynthetic genes.

Hepatol Commun. 2023-10-1

[9]
Computational pathology in cancer diagnosis, prognosis, and prediction - present day and prospects.

J Pathol. 2023-8

[10]
Ductular reaction-associated neutrophils promote biliary epithelium proliferation in chronic liver disease.

J Hepatol. 2023-10

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