Gupta Vikas, Sehrawat Tejasav S, Pinzani Massimo, Strazzabosco Mario
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.
Gastroenterology. 2025 Apr;168(4):675-690. doi: 10.1053/j.gastro.2024.07.044. Epub 2024 Sep 7.
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.
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。
Am J Physiol Cell Physiol. 2024-3-1
Int J Clin Exp Pathol. 2015-10-1
Dig Dis. 2015
Am J Physiol Gastrointest Liver Physiol. 2019-10-21
Nat Commun. 2022-8-30
Am J Physiol Gastrointest Liver Physiol. 2017-8-1
Cell Mol Gastroenterol Hepatol. 2025-6-21
N Engl J Med. 2024-2-29
N Engl J Med. 2024-2-29
Expert Opin Ther Targets. 2023