Targeting Alveolar Repair in Idiopathic Pulmonary Fibrosis

• Published in: American journal of respiratory cell and molecular biology Vol. 65; no. 4; pp. 347 - 365
• Main Authors: Ptasinski, Victoria A., Stegmayr, John, Belvisi, Maria G., Wagner, Darcy E., Murray, Lynne A.
• Format: Journal Article
• Language: English
• Published: New York American Thoracic Society 01.10.2021
• Subjects: Aging; Alveoli; Apoptosis; Cells; Clinical Medicine; Epithelium; Extracellular matrix; Fibrosis; Klinisk medicin; Lung cancer; Lung diseases; Lungmedicin och allergi; Medical and Health Sciences; Medicin och hälsovetenskap; Progenitor cells; Pulmonary fibrosis; Respiratory Medicine and Allergy; Senescence; Stem cells; Studies; Translational Review; Wound healing
• ISSN: 1044-1549; 1535-4989; 1535-4989
• DOI: 10.1165/rcmb.2020-0476TR

Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease with limited therapeutic options. Current evidence suggests that IPF may be initiated by repeated epithelial injuries in the distal lung, which are followed by abnormal wound healing responses that occur because of intrinsic and extrinsic factors. Mechanisms contributing to chronic damage of the alveolar epithelium in IPF include dysregulated cellular processes such as apoptosis, senescence, abnormal activation of the developmental pathways, aging, and genetic mutations. Therefore, targeting the regenerative capacity of the lung epithelium is an attractive approach in the development of novel therapies for IPF. Endogenous lung regeneration is a complex process involving coordinated cross-talk among multiple cell types and reestablishment of a normal extracellular matrix environment. This review will describe the current knowledge of reparative epithelial progenitor cells in the alveolar region of the lung and discuss potential novel therapeutic approaches for IPF, focusing on endogenous alveolar repair.