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The leading role of epithelial cells in the pathogenesis of idiopathic pulmonary fibrosis

机译：上皮细胞在特发性肺纤维化发病机制中的主要作用

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摘要

Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive and devastating interstitial lung disease of unknown etiology, where the normal lung architecture is lost and replaced by fibrotic tissue leading to an irreversible and progressive respiratory insufficiency. Historically, IPF was considered a chronic inflammatory disorder, which gradually progressed to established fibrosis. However, strong clinical and experimental evidence indicates that the disease represents an epithelial-driven disorder which results from a complex interplay of genetic and environmental risk factors, aging-associated processes and a profibrotic epigenetic reprogramming. The convergence of these factors results in the aberrant activation of epithelial cells that initiate the development of the disease, producing virtually all the mediators that participate in the migration, proliferation and activation of fibroblasts, their differentiation to myofibroblasts and the excessive and chaotic secretion of extracellular matrix proteins. Although progress has been made in understanding the causes and consequences of this abnormal behavior of distal airways and alveolar epithelium, the mechanisms that initiate and perpetuate the vicious circle of multidirectional abnormal communications between the epithelium and fibroblasts and other resident cells have not been elucidated. In this review, we discuss the role of epithelial cells and the mechanisms underlying the fibrotic response in IPF, and highlight some promising therapeutic targets for these cells.
机译：特发性肺纤维化（IPF）是一种无情的渐进性和毁灭性的不良肺病，其未知病因，其中正常的肺部结构丢失并被纤维化组织所取代，导致不可逆和渐进的呼吸功能不全。从历史上看，IPF被认为是一种慢性炎症疾病，逐渐进展到建立纤维化。然而，强烈的临床和实验证据表明该疾病是一种上皮驱动的疾病，由遗传和环境危险因素，老化相关过程和血压表观遗传重编程的复杂相互作用。这些因素的收敛导致起始的上皮细胞的异常激活，所述上皮细胞发动疾病的发展，几乎所有参与成纤维细胞的迁移，增殖和激活的所有介质，它们与肌纤维素细胞的分化和细胞外的过量和混沌分泌基质蛋白。虽然在理解远端气道和肺泡上皮的这种异常行为的原因和后果方面取得了进展，但尚未阐明启动和延伸上皮和成纤维细胞之间的多向异常通信的恶性循环的机制。在本综述中，我们讨论了上皮细胞和IPF中纤维化反应的机制的作用，并突出了这些细胞的一些有希望的治疗靶标。

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