Why do patients get idiopathic pulmonary fibrosis? Current concepts in the pathogenesis of pulmonary fibrosis

BMC Medicine

Martin Kolb¹  Pierre-Simon Bellaye¹ 

[1]Department of Pathology and Molecular Medicine, McMaster University Hamilton, Hamilton, Canada

关键词: Wound healing;    Myofibroblast;    Lung injury;    Idiopathic pulmonary fibrosis;    Fibrogenesis;    Extracellular matrix;   

Others  :  1227206
DOI  :  10.1186/s12916-015-0412-6

 received in 2015-06-24, accepted in 2015-06-25,  发布年份 2015

【 摘 要 】

Idiopathic pulmonary fibrosis (IPF) is a devastating lung disease of unknown origin. Recent findings suggest that IPF results from multiple factors that eventually lead to interstitial lung injury. In the pathogenesis it is likely that complex relationships between genetic predispositions, environmental exposures, and lung infections promote the fibrotic processes causing IPF; it is this complexity and the multiplicity of causes that make the population and clinical course of IPF so heterogeneous. Thus, it is clear that one common factor driving IPF pathogenesis in all patients would be far too simplified of an understanding. In recent years, efforts have been made in finding therapeutic strategies that target disease progression rather than disease onset. The biochemical composition and abnormal stiffness of the matrix might be crucial in controlling the cellular phenotype in fibrotic lungs that promotes disease progression and persistence. Though there has been substantial progress in the IPF field in recent years, much more work is required in order to improve the prognosis associated with this disease.

【 授权许可】

   
2015 Bellaye and Kolb.

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