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Molecular Targets in Pulmonary Fibrosis*: The Myofibroblast in Focus

Chris J. Scotton, PhD; Rachel C. Chambers, PhD
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*From the Centre for Respiratory Research, University College London, Rayne Institute, London, UK.

Correspondence to: Rachel Chambers, PhD, Centre for Respiratory Research, University College London, Rayne Institute, 5 University St, London WC1E 6JJ, UK; e-mail: r.chambers@ucl.ac.uk



Chest. 2007;132(4):1311-1321. doi:10.1378/chest.06-2568
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Idiopathic pulmonary fibrosis (IPF) is one of a group of interstitial lung diseases that are characterized by excessive matrix deposition and destruction of the normal lung architecture. Long-term survival of IPF patients is poor, with a 5-year survival rate of only 20%. Despite a lack of evidence-based benefit, IPF has historically been treated with corticosteroids and/or cytotoxic agents such as prednisone. Given the poor efficacy of these drugs, novel therapeutic strategies are required for the management of IPF. This demands a better understanding of the molecular mechanisms underlying the pathogenesis and progression of this disease. The primary effector cell in fibrosis is the myofibroblast; these cells are highly synthetic for collagen, have a contractile phenotype, and are characterized by the presence of α-smooth muscle actin stress fibers. They may be derived by activation/proliferation of resident lung fibroblasts, epithelial-mesenchymal differentiation, or recruitment of circulating fibroblastic stem cells (fibrocytes). From a therapeutic viewpoint, interfering with the pathways that lead to myofibroblast expansion should be of considerable benefit in the treatment of IPF. This review will highlight some of the key molecules involved in this process and the clinical trials that have ensued.

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