Molecular targets in pulmonary fibrosis: the myofibroblast in focus

CJ Scotton, RC Chambers - Chest, 2007 - Elsevier
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 …