Pathogenesis of IPF: Is abnormal repair of epithelial damage involved in the basic pathogenesis of this disease?

Abstract

The molecular pathogenesis in IPF is not fully understood. However, epithelial injury and subsequent aberrant wound healing, rather than chronic inflammation, are thought to play central roles in the recent hypothesis. Alveolar epithelial cells predisposed with genetic mutations may involve in the abnormal responses subsequent to injury. Growth factors, such as transforming growth factor- ß and platelet-derived growth factors, are critical mediators that control the growth and differentiation of lung fibroblasts. The novel topics in pulmonary fibrosis include the origin of lung fibroblasts, alveolar epithelial integrity, and resolution of extracellular matrixes. The further studies are required to clarify the mechanisms involved in the fibrogenesis in the lungs of IPF.