Journal
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY
Volume 306, Issue 11, Pages C987-C996Publisher
AMER PHYSIOLOGICAL SOC
DOI: 10.1152/ajpcell.00321.2013
Keywords
epithelium; lung fibrosis; myofibroblast
Categories
Funding
- NHLBI NIH HHS [R01 HL060539, P01 HL108793] Funding Source: Medline
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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.
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