Despite reductions in mortality in cancer and cardiovascular disease, mortality rates for chronic lung diseases including interstitial lung disease have remained unaffected over the last decade. Interstitial lung disease (ILD) is a heterogeneous group of restrictive lung disorders that are characterized by the deposition of fibrotic tissue in the lungs, which progressively reduces gas exchange and ultimately leads to death from respiratory failure. The most common presentation of lung fibrosis is Idiopathic pulmonary fibrosis (IPF). However, lung fibrosis is also found in disorders such as scleroderma, sarcoidosis, and cystic fibrosis.
Despite the emergence of two novel compounds for the treatment of lung fibrosis, lung transplantation remains the only “curative” option1 despite a low 5-year mean survival rate following transplantation. This highlights the need to better understand the mechanisms that lead to pulmonary fibrosis to develop new therapies.
The etiology of ILD is complex, implicating mutations in genes involved in the maintenance of telomere length, expression of cilium-associated genes, proteostatic dysregulation and enhanced cellular senescence. Central to the development of lung fibrosis is the reprogramming of epithelial and mesenchymal cells and immune cell dysregulation resulting in an enhanced extracellular matrix (ECM) deposition and lung remodeling. A central process that drives the development of lung fibrosis is a recurrent subclinical injury to the epithelium and subsequent recapitulation of embryonic developmental programs. Risk factors for the development of lung fibrosis include advanced age, exposure to environmental toxicants, such as mining dust and genetic predisposition. However, the interplay between seemingly disparate pathways: developmental gene expression and cellular senesce in fibrotic lung diseases are not fully understood. The goal of this proposal is to identify novel developmental and cellular senesce processes that contribute to lung remodeling in ILD.
This Research Topic welcomes original research manuscripts that identify novel embryonic or senescence mediators and their contribution to lung fibrosis, utilizing in vitro and/or in vivo approaches. We also welcome manuscripts that focus on the pathophysiology of specific ILDs including but not limited to IPF, scleroderma, sarcoidosis, or cystic fibrosis, as long as they focus on developmental or senescence programs.
Despite reductions in mortality in cancer and cardiovascular disease, mortality rates for chronic lung diseases including interstitial lung disease have remained unaffected over the last decade. Interstitial lung disease (ILD) is a heterogeneous group of restrictive lung disorders that are characterized by the deposition of fibrotic tissue in the lungs, which progressively reduces gas exchange and ultimately leads to death from respiratory failure. The most common presentation of lung fibrosis is Idiopathic pulmonary fibrosis (IPF). However, lung fibrosis is also found in disorders such as scleroderma, sarcoidosis, and cystic fibrosis.
Despite the emergence of two novel compounds for the treatment of lung fibrosis, lung transplantation remains the only “curative” option1 despite a low 5-year mean survival rate following transplantation. This highlights the need to better understand the mechanisms that lead to pulmonary fibrosis to develop new therapies.
The etiology of ILD is complex, implicating mutations in genes involved in the maintenance of telomere length, expression of cilium-associated genes, proteostatic dysregulation and enhanced cellular senescence. Central to the development of lung fibrosis is the reprogramming of epithelial and mesenchymal cells and immune cell dysregulation resulting in an enhanced extracellular matrix (ECM) deposition and lung remodeling. A central process that drives the development of lung fibrosis is a recurrent subclinical injury to the epithelium and subsequent recapitulation of embryonic developmental programs. Risk factors for the development of lung fibrosis include advanced age, exposure to environmental toxicants, such as mining dust and genetic predisposition. However, the interplay between seemingly disparate pathways: developmental gene expression and cellular senesce in fibrotic lung diseases are not fully understood. The goal of this proposal is to identify novel developmental and cellular senesce processes that contribute to lung remodeling in ILD.
This Research Topic welcomes original research manuscripts that identify novel embryonic or senescence mediators and their contribution to lung fibrosis, utilizing in vitro and/or in vivo approaches. We also welcome manuscripts that focus on the pathophysiology of specific ILDs including but not limited to IPF, scleroderma, sarcoidosis, or cystic fibrosis, as long as they focus on developmental or senescence programs.
