title: Cellular mechanisms of tissue fibrosis. 7. New insights into the cellular mechanisms of pulmonary fibrosis.
abstract: 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.
authors.affiliation: Division of Pulmonary, Allergy, and Critical Care Medicine, Duke University, Durham, North Carolina; and.
authors.forename: Christina E
authors.initials: CE
authors.lastname: Barkauskas
authors.affiliation: Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, California paul.noble@cshs.org.
authors.forename: Paul W
authors.initials: PW
authors.lastname: Noble
mesh_terms: D000818:Animals; D005347:Fibroblasts / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D011650:Pulmonary Alveoli / Q000473:pathology; D011658:Pulmonary Fibrosis / Q000378:metabolism / Q000473:pathology*; D020545:Respiratory Mucosa / Q000378:metabolism / Q000473:pathology*
keywords: epithelium; lung fibrosis; myofibroblast
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