Cell-based therapy for idiopathic pulmonary fibrosis

Qi Lu, Ahmed H. K. El-Hashash


Idiopathic pulmonary fibrosis (IPF) is an example of interstitial lung diseases that is characterized by chronic, progressive, and fibrotic lung injuries. During lung fibrosis, normal healthy lung tissues are replaced by remarkably destroyed alveolar architecture and altered extracellular cell matrix. These changes eventually cause severe disruption of the tightly-controlled gas exchange process and reduction of lung compliance that ultimately lead to both respiratory failure and death. In the last decade, progress has been made toward understanding the pathogenesis of pulmonary fibrosis, and two novel disease-modifying therapies were approved. However, finding more effective treatments for pulmonary fibrosis is still a challenge, with its incidence continues to increase globally, which is associated with significantly high mortality, morbidity and economical healthcare burden. Different stem cell types have recently emerged as a promising therapy for human diseases, including lung fibrosis, with numerous studies on the identification, characterization, proliferation and differentiation of stem cells. A large body of both basic and pre-clinical research on stem cells has been recently translated to patient care worldwide. Herein, we review recent advances in our understanding of the pathophysiology of IPF, and types of cells used in IPF cell-based therapies, including alveolar and mixed lung epithelial cells, different stem cell types (MSCs, ADSCs, IPSCs…etc.), endogenous lung tissue-specific stem cells, and circulating endothelial progenitors (EPCs). We also discuss recent studies on the applications of these cells in IPF therapy and their delivery routes, effective doses for cell therapy, and timing of delivery. Finally, we discuss attractive recent and current clinical trials conducted on cell-based therapy for IPF.