The contribution of animal models to understanding the role of the immune system in human idiopathic pulmonary fibrosis

Tylah Miles; Gerard F Hoyne; Darryl A Knight; Mark W Fear; Steven E Mutsaers; Cecilia M Prêle

doi:10.1002/cti2.1153

The contribution of animal models to understanding the role of the immune system in human idiopathic pulmonary fibrosis

Clin Transl Immunology. 2020 Jul 27;9(7):e1153. doi: 10.1002/cti2.1153. eCollection 2020.

Authors

Tylah Miles^{1

2}, Gerard F Hoyne^{3

4}, Darryl A Knight^{5

6}, Mark W Fear⁷, Steven E Mutsaers^{1

2

3}, Cecilia M Prêle^{2

3

8}

Affiliations

¹ Institute for Respiratory Health Nedlands WA Australia.
² Centre for Respiratory Health School of Biomedical Sciences University of Western Australia Nedlands WA Australia.
³ Centre for Cell Therapy and Regenerative Medicine School of Biomedical Sciences University of Western Australia Nedlands WA Australia.
⁴ School of Health Sciences University of Notre Dame Australia Fremantle WA Australia.
⁵ Providence Health Care Research Institute Vancouver BC Canada.
⁶ University of British Columbia Vancouver BC Canada.
⁷ Burn Injury Research Unit School of Biomedical Sciences The University of Western Australia Crawley WA Australia.
⁸ Ear Science Institute Australia Nedlands WA Australia.

Abstract

Pulmonary fibrosis occurs in a heterogeneous group of lung disorders and is characterised by an excessive deposition of extracellular matrix proteins within the pulmonary interstitium, leading to impaired gas transfer and a loss of lung function. In the past 10 years, there has been a dramatic increase in our understanding of the immune system and how it contributes to fibrogenic processes within the lung. This review will compare some of the models used to investigate the pathogenesis and treatment of pulmonary fibrosis, in particular those used to study immune cell pathogenicity in idiopathic pulmonary fibrosis, highlighting their advantages and disadvantages in dissecting human disease.

Keywords: animal models; bleomycin; fibrogenesis; inflammation; innate and adaptive immune system.