Immunobiology of Pulmonary Fibrosis

Samik Bindu¹Hadida Yasmin¹Uttam Barman¹Subhradip Pandit¹Khaled Masmoudi²Uday Kishore^2*

• ¹Cooch Behar Panchanan Barma University, Cooch Behar, India
• ²Biosciences, United Arab Emirates University, Al-Ain, United Arab Emirates

Pulmonary fibrosis (PF), an interstitial lung disease (ILD), is characterized by progressive thickening and scarring of the lung tissue associated with shortness of breath, decreased vital capacity, and respiratory failure due to the inability to expand and contract the lungs during inspiration with severe morbidity and mortality. The median estimated survival is 2-5 years after the day of diagnosis. Current understanding of how the disease initiates and progresses suggests a set of complex mechanisms involving a combination of genetic vulnerability, aging processes, and environmental factors. Mechanistically, the damage of the alveolar epithelial cells (AECs) (type I and type II), followed by recruitment of immune cells and transdifferentiation of fibroblast to myofibroblast play a crucial role in the initiation of a prolonged wound healing response in lungs which eventually leads to fibrosis if healing gets awry. Here, we have systematically reviewed the role of innate and adaptive immunity, as well as interactions of lung parenchyma cells together with the immune cells, cytokines, chemokines and other mediators in the context of pulmonary fibrosis. We have also discussed how the selection of pre-clinical models is important for the understanding of the disease and successful clinical trial outcome.