The DOI matches its Crossref record; 214 of the 214 references it cites were found in a registry or an index.
Inflammation and immunity in IPF pathogenesis and treatment
Open accessAbstract
Idiopathic pulmonary fibrosis (IPF) is a progressive, and ultimately fatal, chronic interstitial lung disease characterized by enhanced extracellular matrix deposition. Repetitive alveolar epithelial injury triggers the early development of fibrosis. These injuries, in combination with dysregulated wound repair and fibroblast dysfunction, lead to ongoing tissue remodelling and fibrosis seen in end-stage pulmonary fibrosis. Although the exact etiology in IPF is unknown and probably diverse, all stages of fibrosis are accompanied by innate and adaptive immune responses. The role of inflammation as an important component in IPF etiology is controversial and sometimes seen as an epiphenomenon of fibrosis. This view is partly the result of negative multicenter trials of anti-inflammatory drugs for IPF treatment. However, new insights on the role of macrophages, the loss of T-cell and B-cell tolerance leading auto-immune responses in IPF, and the interaction of immune cells with (myo)fibroblasts have led to a slow change of this opinion. Clearly, more insight is needed to integrate basic immune mechanisms into translational research and finally new IPF therapies. In this concise review, we will focus on the role of our innate and adaptive immune system in the initiation and perpetuation of IPF pathobiology. Next, we will discuss how immune responses are influenced by current anti-fibrotic treatments, such as pirfenidone and nintedanib and end with an overview of recent and upcoming therapeutic trials that target and modulate our immune system in patients with IPF.
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Cites
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Gordon, S., & Taylor, P. R. (2005) · Nature Reviews Immunology · doi:10.1038/nri1733 · Cited by 5,190
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Wynn, T. A., Chawla, A., & Pollard, J. W. (2013) · Nature · doi:10.1038/nature12034 · Cited by 4,684
Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis
Richeldi, L., du Bois, R. M., Raghu, G., Azuma, A., Brown, K. K., Costabel, U., Cottin, V., Flaherty, K. R., Hansell, D. M., Inoue, Y., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402584 · Cited by 4,654
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Wynn, T. (2008) · The Journal of Pathology · doi:10.1002/path.2277 · Cited by 4,495
Alternative activation of macrophages: mechanism and functions
Gordon, S., & Martinez, F. O. (2010) · Immunity · doi:10.1016/j.immuni.2010.05.007 · Cited by 4,197
A phase 3 trial of pirfenidone in patients with idiopathic pulmonary fibrosis.
King, T. E., Bradford, W. Z., Castro-Bernardini, S., Fagan, E. A., Glaspole, I., Glassberg, M. K., Gorina, E., Hopkins, P. M., Kardatzke, D., Lancaster, L., et al. (2014) · New England Journal of Medicine · doi:10.1056/nejmoa1402582 · Cited by 3,975
Monocyte Chemoattractant Protein-1 (MCP-1): An Overview
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Cited by
The interplay of fibroblasts, the extracellular matrix, and inflammation in scar formation
Moretti, L., Stalfort, J., Barker, T. H., & Abebayehu, D. (2022) · Journal of Biological Chemistry · doi:10.1016/j.jbc.2021.101530 · Cited by 393
Idiopathic Pulmonary Fibrosis: An Update on Pathogenesis
Mei, Q., Liu, Z., Zuo, H., Yang, Z., & Qu, J. (2022) · Frontiers in Pharmacology · doi:10.3389/fphar.2021.797292 · Cited by 360
Savin, I. A., Zenkova, M. A., & Sen’kova, A. V. (2022) · International Journal of Molecular Sciences · doi:10.3390/ijms232314959 · Cited by 279
Herrmann, F. E., Hesslinger, C., Wollin, L., & Nickolaus, P. (2022) · Frontiers in Pharmacology · doi:10.3389/fphar.2022.838449 · Cited by 137
Idiopathic pulmonary fibrosis.
Koudstaal, T., & Wijsenbeek, M. S. (2023) · La Presse Médicale · doi:10.1016/j.lpm.2023.104166 · Cited by 105
Self-citation
Metadata
- Authors
- Heukels, P., Moor, C. C., von der Thüsen, J. H., Wijsenbeek, M. S., & Kool, M.
- Venue
- Respiratory Medicine
- Publisher
- Elsevier BV
- Published
- 2019-02
- Volume
- 147
- Pages
- 79-91
- ISSN
- 0954-6111 (ISSN-L)
- OpenAlex
- W2909223530
- Cited by
- 517
- References
- 214
- FWCI
- 30.64
- Type
- journal article
- Language
- en
- Open access
- bronze
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- Heukels, P., Moor, C. C., von der Thüsen, J. H., Wijsenbeek, M. S., & Kool, M. (2019). Inflammation and immunity in IPF pathogenesis and treatment. Respiratory Medicine. https://doi.org/10.1016/j.rmed.2018.12.015
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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