Molecular and cellular mechanisms of pulmonary fibrosis

Fibrogenesis & Tissue Repair

Sergei P Atamas¹  Irina G Luzina¹  Nevins W Todd² 

[1] VA Medical Center, Baltimore, MD, USA;Division of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, 110 S. Paca St., Baltimore, MD, 21201, USA

关键词: Coagulation;    Oxidative stress;    Inflammation;    Epithelial cells;    Fibroblasts;    Collagen;    Extracellular matrix;    Idiopathic pulmonary fibrosis;   

Others  :  803702
DOI  :  10.1186/1755-1536-5-11

 received in 2012-04-02, accepted in 2012-06-28,  发布年份 2012

【 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.

【 授权许可】

   
2012 Todd et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

Figure 2.

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