医学
细胞外基质
肺纤维化
纤维化
特发性肺纤维化
肺
炎症
氧化应激
疾病
病理
癌症研究
免疫系统
免疫学
间充质干细胞
细胞生物学
肌成纤维细胞
机制(生物学)
内科学
生物
作者
Nevins W. Todd,Irina G. Luzina,Sergei P. Atamas
标识
DOI:10.1186/1755-1536-5-11
摘要
Pulmonary fibrosis is a chronic lung disease characterized by excessive accumulation of extracellular matrix (ECM) and remodeling of the lung architecture. Idiopathic pulmonary fibrosis is considered the most common and severe form of the disease, with a median survival of approximately three years and no proven effective therapy. Despite the fact that effective treatments are absent and the precise mechanisms that drive fibrosis in most patients remain incompletely understood, an extensive body of scientific literature regarding pulmonary fibrosis has accumulated over the past 35 years. In this review, we discuss three broad areas which have been explored that may be responsible for the combination of altered lung fibroblasts, loss of alveolar epithelial cells, and excessive accumulation of ECM: inflammation and immune mechanisms, oxidative stress and oxidative signaling, and procoagulant mechanisms. We discuss each of these processes separately to facilitate clarity, but certainly significant interplay will occur amongst these pathways in patients with this disease.
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