Email updates

Keep up to date with the latest news and content from Fibrogenesis & Tissue Repair and BioMed Central.

Open Access Highly Accessed Review

Molecular and cellular mechanisms of pulmonary fibrosis

Nevins W Todd123*, Irina G Luzina12 and Sergei P Atamas12

Author Affiliations

1 Department of Medicine, University of Maryland School of Medicine, Baltimore, MD, USA

2 VA Medical Center, Baltimore, MD, USA

3 Division of Pulmonary and Critical Care Medicine, University of Maryland School of Medicine, 110 S. Paca St., Baltimore, MD, 21201, USA

For all author emails, please log on.

Fibrogenesis & Tissue Repair 2012, 5:11  doi:10.1186/1755-1536-5-11

Published: 23 July 2012

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.

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