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. 2012 Jul 23;5(1):11.
doi: 10.1186/1755-1536-5-11.

Molecular and cellular mechanisms of pulmonary fibrosis

Nevins W Todd  1 , Irina G Luzina, Sergei P Atamas
Affiliations

Molecular and cellular mechanisms of pulmonary fibrosis

Nevins W Todd et al. Fibrogenesis Tissue Repair. .

Abstract

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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Figures

Figure 1
Figure 1
Cumulative number of publications using PubMed searches for articles on pulmonary fibrosis, excluding cystic fibrosis. Search strategy (open circles) was ‘((lung OR pulmonary) AND (fibrosis OR fibrotic) AND english [la] AND hasabstract) NOT cystic’, or when referred to in the title, search strategy (closed circles) was ‘((lung [ti] OR pulmonary [ti]) AND (fibrosis [ti] OR fibrotic [ti]) AND english [la] AND hasabstract) NOT cystic [ti]’. These cumulative numbers most likely underestimate the realistic breadth of literature on the topic, as pulmonary fibrosis is often described using various terms (fibrosing alveolitis, interstitial lung disease), and a large body of literature has focused on cellular and molecular responses in cell culture (fibroblasts, epithelial, endothelial, and inflammatory cells) without mentioning pulmonary fibrosis in the title or the abstract.
Figure 2
Figure 2
Schematic diagram representing three broad mechanisms (inflammation, oxidative stress, and coagulation disturbances) that alone or in combination may be responsible for alterations in mesenchymal cells, epithelial cells, and extracellular matrix (ECM) that result in pulmonary fibrosis following lung injury.
See this image and copyright information in PMC

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