Cellular mechanisms of tissue fibrosis. 7. New insights into the cellular mechanisms of pulmonary fibrosis

CE Barkauskas, PW Noble - American Journal of …, 2014 - journals.physiology.org
CE Barkauskas, PW Noble
American Journal of Physiology-Cell Physiology, 2014journals.physiology.org
Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by severe and
progressive scar formation in the gas-exchange regions of the lung. Despite years of
research, therapeutic treatments remain elusive and there is a pressing need for deeper
mechanistic insights into the pathogenesis of the disease. In this article, we review our
current knowledge of the triggers and/or perpetuators of pulmonary fibrosis with special
emphasis on the alveolar epithelium and the underlying mesenchyme. In doing so, we raise …
Idiopathic pulmonary fibrosis (IPF) is a devastating disease characterized by severe and progressive scar formation in the gas-exchange regions of the lung. Despite years of research, therapeutic treatments remain elusive and there is a pressing need for deeper mechanistic insights into the pathogenesis of the disease. In this article, we review our current knowledge of the triggers and/or perpetuators of pulmonary fibrosis with special emphasis on the alveolar epithelium and the underlying mesenchyme. In doing so, we raise a number of questions highlighting critical voids and limitations in our current understanding and study of this disease.
American Physiological Society