Idiopathic pulmonary fibrosis
Idiopathic pulmonary fibrosis
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DOI：
10.1201/b13341-74
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特发性肺纤维化是一种进行性肺部疾病，预后不良，没有有效的治疗方法。虽然过度沉积的细胞外基质，结合证据的反复损伤肺泡上皮细胞，是一个迫切需要了解这些过程更好地在分子水平上，从而确定潜在的治疗目标，在这种棘手的疾病。本综述考虑了最近发表在Thorax和其他地方的一些进展，这些进展提高了我们对特发性肺纤维化病理生理学的理解，使用来自人类细胞和组织以及肺纤维化动物模型的数据。这些研究特别关注肺泡上皮细胞的命运和纤维生成机制，并确定与肺癌和肺动脉高压等共存疾病共有的机制途径。疾病进展和预后的生理生物标志物的概念也进行了讨论。
Idiopathic pulmonary fibrosis is a progressive lung disease that carries a poor prognosis and for which there are no effective therapies. Although the excessive deposition of extracellular matrix, combined with evidence of recurrent injury to the alveolar epithelium, are well-described there is a pressing need to understand these processes better at a molecular level and thus to identify potential therapeutic targets in this intractable disease. This review considers some recent advances published in Thorax and elsewhere that have improved our understanding of the pathophysiology of idiopathic pulmonary fibrosis, using data both from human cells and tissue and from animal models of pulmonary fibrosis. The studies particularly address the fate of the alveolar epithelial cell and mechanisms of fibrogenesis, and identify mechanistic pathways shared with co-existing conditions such as lung cancer and pulmonary hypertension. The concepts of physiological biomarkers of disease progression and prognosis are also discussed.