Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive- fibrosing disease characterized by extensive deposition of extracellular matrix (ECM), scarring of the lung parenchyma. Despite increased awareness of IPF, etiology and physiological mechanism of IPF are unclear. Therefore, preclinical model will require relevant and recapitulative features of IPF. Recently, pluripotent stem cells (PSC)-based organoid studies are emerging as an alternative approach able to recapitulate tissue architecture with remarkable fidelity. Moreover, these biomimetic tissue models can be served to investigate the mechanisms of diverse disease progression. In this review, we will overview the current organoids technology for human disease modeling including lung organoids for IPF.

中文翻译：

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特发性肺纤维化的器官模型。

特发性肺纤维化（IPF）是一种慢性进行性纤维化疾病，其特征是细胞外基质（ECM）大量沉积，肺实质形成瘢痕。尽管人们对IPF的认识有所提高，但IPF的病因和生理机制尚不清楚。因此，临床前模型将需要IPF的相关功能和概括功能。最近，基于多能干细胞（PSC）的类器官研究正在出现，它是一种能够以出色的逼真度概括组织结构的替代方法。此外，这些仿生组织模型可用于研究多种疾病进展的机制。在这篇综述中，我们将概述用于人类疾病建模的当前类器官技术，包括IPF的肺类器官。