Anne Burnett Marion School of Medicine, Texas Christian University, Fort Worth, TX 76129, USA.
Department of Biomedical Engineering, University of North Texas, Denton, TX 76207, USA.
Int J Mol Sci. 2024 Nov 1;25(21):11751. doi: 10.3390/ijms252111751.
Idiopathic pulmonary fibrosis (IPF) is a lethal disorder characterized by relentless progression of lung fibrosis that causes respiratory failure and early death. Currently, no curative treatments are available, and existing therapies include a limited selection of antifibrotic agents that only slow disease progression. The development of novel therapeutics has been hindered by a limited understanding of the disease's etiology and pathogenesis. A significant challenge in developing new treatments and understanding IPF is the lack of in vitro models that accurately replicate crucial microenvironments. In response, three-dimensional (3D) in vitro models have emerged as powerful tools for replicating organ-level microenvironments seen in vivo. This review summarizes the state of the art in advanced 3D lung models that mimic many physiological and pathological processes observed in IPF. We begin with a brief overview of conventional models, such as 2D cell cultures and animal models, and then explore more advanced 3D models, focusing on lung-on-a-chip systems. We discuss the current challenges and future research opportunities in this field, aiming to advance the understanding of the disease and the development of novel devices to assess the effectiveness of new IPF treatments.
特发性肺纤维化（IPF）是一种致命疾病，其特征是肺纤维化的无情进展，导致呼吸衰竭和早逝。目前，尚无治愈方法，现有的治疗方法包括有限的抗纤维化药物选择，这些药物只能减缓疾病进展。由于对疾病的病因和发病机制的了解有限，新型治疗药物的开发受到阻碍。开发新疗法和了解 IPF 的一个重大挑战是缺乏能够准确复制关键微环境的体外模型。为应对这一挑战，三维（3D）体外模型已成为复制体内观察到的器官水平微环境的有力工具。本综述总结了模拟 IPF 中观察到的许多生理和病理过程的先进 3D 肺模型的最新进展。我们首先简要概述了传统模型，如 2D 细胞培养和动物模型，然后探讨了更先进的 3D 模型，重点介绍了肺芯片系统。我们讨论了该领域当前的挑战和未来的研究机会，旨在推进对该疾病的理解和开发新设备以评估新型 IPF 治疗方法的有效性。
