Editorial: Progressive fibrosing interstitial lung disease: from bench to bedside
Julien Guiot1,2* 
Makon-Sébastien Njock1,2*- 1Department of Respiratory Medicine, University Hospital of Liège, Liège, Belgium
- 2Laboratory of Pneumology, GIGA Research Center, University of Liège, Liège, Belgium
Editorial on the Research Topic
Progressive fibrosing interstitial lung disease: from bench to bedside
Introduction
Pulmonary fibrosis is a debilitating and potentially progressive lung disease characterized by the excessive accumulation of fibrotic tissue within the lung parenchyma, leading to impaired gas exchange and respiratory failure. Despite significant advances in our understanding of the pathogenesis and management of pulmonary fibrosis, the disease remains a therapeutic challenge. To date, the gold standard for the diagnosis of progressive pulmonary fibrosis (PPF) is a holistic evaluation through multimodal assessment including the analysis of clinical symptoms, pulmonary function test (PFT), histopathological evaluation and high-resolution computed tomography (HRCT) of the chest (1).
This editorial aims to shed light on the current state of research and clinical approaches in the field of PPF, emphasizing the need of early biomarkers helping clinicians to identify PPF at the earliest stage and also new potential therapeutic targets aiming to stop the uncontrolled fibrotic process. This Research Topic currently includes 15 original research articles on the diagnosis/prognosis of PPF, based on the identification of news biomarkers, multi-scale analysis of clinical informations and parameters, which results on the development of prediction models. These research articles are from interdisciplinary collaboration (clinicians, researchers and industry partners) and emerging technologies. All contributions to this Research Topic focus on one or more of the research areas highlighted above.
Clinical management
Despite the recent progress in understanding the molecular mechanisms of pulmonary fibrosis, the clinical management of patients with progressive disease remains a complex challenge. Indeed, as stated in the recent guidelines (2), clinicians have to wait the reduction of PFT and/or a PPF based on imaging analysis. ATS/ERS recommendations state that diagnostic criteria for PPF are a combination of two criteria including: worsening symptoms over time, HRCT-proven fibrotic progression and/or an absolute decline from baseline in FVC (≥5%) or DLCO (≥10%) over 1 year of follow-up.
In a review article, Stanel and Rivera-Ortega have discussed about the perspectives in early diagnosis and monitoring for progressive fibrosing interstitial lung diseases (PF-ILD). In a retrospective study, Chiu et al. have examined the prognostic relevance of PPF definitions, and shown that it did not differ between simplified PPF, INBUILD and ATS/ERS/JRS/ALAT 2022 criteria. A recent study by Takei et al. highlights the need to consider an evaluation of health-related quality of life when assessing PPF in patients with idiopathic pulmonary fibrosis.
Currently available therapies, such as antifibrotic drugs, primarily target the fibrotic process but have limited efficacy in preventing early disease progression. As a result, a comprehensive and personalized approach to patient care is required. Clinical trials evaluating combination therapies, precision medicine approaches, and novel drug delivery systems hold promise for improving patient outcomes and quality of life (Wu et al.).
Emerging technologies and biomarkers
The advent of advanced technologies has revolutionized the field of pulmonary fibrosis research. High-throughput genomics, proteomics, and metabolomics have allowed researchers to identify novel biomarkers associated with disease progression and prognosis. These biomarkers not only aid in early diagnosis but also serve as valuable tools for monitoring treatment response and predicting outcomes. Additionally, innovative imaging techniques, such as HRCT and functional lung imaging, contribute to a more precise assessment of disease severity and progression (3).
With this topic, several studies have developed predictive/prognostic model for PF-ILD based on machine learning algorithms by combining clinical informations (Shao et al.; Lee et al.; Zhang et al.; Niu et al.).
Translational research
Translational research acts as the critical link between bench research and clinical practice, facilitating the transition of promising experimental findings into real-world applications. In the context of PPF, translational studies have demonstrated the efficacy of several novel therapeutic interventions in preclinical models (4). From anti-fibrotic drugs to gene and cell-based therapies, these advancements offer hope for the development of effective treatments that can halt or even reverse disease progression.
Bridging the gap between bench and bedside
The journey from bench to bedside is essential in translating scientific discoveries into effective clinical interventions. Bench research plays a pivotal role in unraveling the underlying molecular mechanisms driving PPF. Investigating key cellular pathways, such as epithelial-mesenchymal transition (EMT), myofibroblast activation, and dysregulated immune responses, has contributed significantly to our understanding of disease pathogenesis (5–7). Fundamental discoveries led to the identification of potential therapeutic targets, paving the way for novel treatment strategies.
Collaboration and data sharing
To accelerate progress in the field of PPF, collaboration and data sharing among researchers, clinicians, and industry partners are of paramount importance. Sharing research findings, clinical data, and biological samples through established networks and databases can foster synergistic efforts and enable more comprehensive analyses (Tomassetti et al.; Quan et al.). Moreover, collaborative efforts facilitate the validation of preclinical research findings in diverse patient populations, ultimately leading to more robust and reliable clinical recommendations.
Importance of patient advocacy and support
Lastly, it is crucial to acknowledge the invaluable role of patient advocacy groups and support networks in raising awareness about PPF. These organizations provide a platform for patients, caregivers, and researchers to collaborate, share experiences, and advocate for improved access to care, research funding, and better overall understanding of the disease. By amplifying patient voices and perspectives, we can drive meaningful change in the field and ensure that scientific advancements are translated into tangible benefits for those affected by the disease.
Conclusion
The journey from bench to bedside in the realm of PPF research holds immense potential for advancing our understanding and treatment of this debilitating disease of high unmet medical need. Through the integration of bench discoveries, translational research, and innovative clinical approaches, we can bridge the gap between scientific knowledge and patient care. By fostering collaboration, embracing emerging technologies, and prioritizing patient advocacy, we can work toward a future where PPF is no longer an insurmountable challenge, but a conquerable condition.
Author contributions
JG: Writing—original draft, Writing—review and editing. M-SN: Writing—original draft, Writing—review and editing.
Funding
This study was supported by the Centre Hospitalier Universitaire de Liège (CHU Liège).
Acknowledgments
The authors would like to thank all co-editors for their contribution.
Conflict of interest
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
Publisher's note
All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.
Abbreviations
EMT, epithelial-mesenchymal transition; HRCT, high-resolution computed tomography; F-ILD, fibrosing interstitial lung diseases; PF-ILD, progressive fibrosing interstitial lung diseases; PFT, pulmonary function test; PPF, progressive pulmonary fibrosis.
References
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2. Raghu G, Remy-Jardin M, Richeldi L, Thomson CC, Inoue Y, Johkoh T, et al. Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. (2022) 205:e18–47. doi: 10.1164/rccm.202202-0399ST
3. Topff L, Sánchez-García J, López-González R, Pastor AJ, Visser JJ, Huisman M, et al. A deep learning-based application for COVID-19 diagnosis on CT: the imaging COVID-19 AI initiative. PLoS ONE. (2023) 18:e0285121. doi: 10.1371/journal.pone.0285121
4. Zhao M, Wang L, Wang M, Zhou S, Lu Y, Cui H, et al. Targeting fibrosis: mechanisms and clinical trials. Sig Transduct Target Ther. (2022) 7:1–21. doi: 10.1038/s41392-022-01070-3
5. Njock M-S, Guiot J, Henket MA, Nivelles O, Thiry M, Dequiedt F, et al. Sputum exosomes: promising biomarkers for idiopathic pulmonary fibrosis. Thorax. (2019) 74:309–12. doi: 10.1136/thoraxjnl-2018-211897
6. Guiot J, Cambier M, Boeckx A, Henket M, Nivelles O, Gester F, et al. Macrophage-derived exosomes attenuate fibrosis in airway epithelial cells through delivery of antifibrotic miR-142-3p. Thorax. (2020) 75:870–81. doi: 10.1136/thoraxjnl-2019-214077
Keywords: progressive fibrosing interstitial lung disease, fibrotic lung diseases, biomarkers, diagnosis, prognosis, predictive model
Citation: Guiot J and Njock M-S (2023) Editorial: Progressive fibrosing interstitial lung disease: from bench to bedside. Front. Med. 10:1277909. doi: 10.3389/fmed.2023.1277909
Received: 15 August 2023; Accepted: 23 August 2023;
Published: 01 September 2023.
Edited and reviewed by: Dawei Yang, Fudan University, China
Copyright © 2023 Guiot and Njock. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
*Correspondence: Julien Guiot, j.guiot@chuliege.be; Makon-Sébastien Njock, ms.njock@chuliege.be