Version 1
: Received: 16 July 2024 / Approved: 17 July 2024 / Online: 17 July 2024 (12:33:13 CEST)
How to cite:
Jenkinson, F.; Rodgers, A. M.; Bell, R. L.; Small, D.; Schock, B. C.; Chaudhuri, N.; Ingram, R. J. Idiopathic Pulmonary Fibrosis: Regulation, Cytokine Signaling, and Gaps in Our Knowledge. Preprints2024, 2024071435. https://doi.org/10.20944/preprints202407.1435.v1
Jenkinson, F.; Rodgers, A. M.; Bell, R. L.; Small, D.; Schock, B. C.; Chaudhuri, N.; Ingram, R. J. Idiopathic Pulmonary Fibrosis: Regulation, Cytokine Signaling, and Gaps in Our Knowledge. Preprints 2024, 2024071435. https://doi.org/10.20944/preprints202407.1435.v1
Jenkinson, F.; Rodgers, A. M.; Bell, R. L.; Small, D.; Schock, B. C.; Chaudhuri, N.; Ingram, R. J. Idiopathic Pulmonary Fibrosis: Regulation, Cytokine Signaling, and Gaps in Our Knowledge. Preprints2024, 2024071435. https://doi.org/10.20944/preprints202407.1435.v1
APA Style
Jenkinson, F., Rodgers, A. M., Bell, R. L., Small, D., Schock, B. C., Chaudhuri, N., & Ingram, R. J. (2024). Idiopathic Pulmonary Fibrosis: Regulation, Cytokine Signaling, and Gaps in Our Knowledge. Preprints. https://doi.org/10.20944/preprints202407.1435.v1
Chicago/Turabian Style
Jenkinson, F., Nazia Chaudhuri and Rebecca Jo Ingram. 2024 "Idiopathic Pulmonary Fibrosis: Regulation, Cytokine Signaling, and Gaps in Our Knowledge" Preprints. https://doi.org/10.20944/preprints202407.1435.v1
Abstract
Idiopathic pulmonary fibrosis [IPF] is a chronic progressive disease characterised by the accumulation of scar tissue in the lung parenchyma. It primarily occurs in middle-aged and elderly adults and results in significant morbidity and mortality worldwide. The disease occurs due to repetitive lung epithelial injury, subsequent fibroblast activation and myofibroblast differentiation, resulting in excessive extracellular matrix deposition. This leads to scar formation and subsequent loss of lung function. Current treatment options for patients with IPF include the two anti-fibrotic drugs, pirfenidone and nintedanib, which can slow disease progression; however, there are currently no known cures for the disease. As such, novel methods and drug targets are warranted. In this review, we provide an up-to-date account of the importance of specific cytokines and the potential role of regulatory immune cells. We discuss their role in the pathogenesis of IPF and address some of the key gaps in knowledge.
Keywords
Idiopathic pulmonary fibrosis; myofibroblast; extracellular matrix; T cells
Subject
Medicine and Pharmacology, Pulmonary and Respiratory Medicine
Copyright:
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
