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Pulmonary Fibrosis: Molecular Pathology, Diagnosis, and Therapeutic Strategies
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Pulmonary Fibrosis: Molecular Pathology, Diagnosis, and Therapeutic Strategies

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: closed (31 July 2024) | Viewed by 7999

Special Issue Editors

Dr. Paolo Cameli

E-Mail Website
Guest Editor
Respiratory Diseases and Lung Transplantation Unit, Department of Medicine, Surgery and Neurosciences, University Hospital of Siena, 53100 Siena, Italy
Interests: interstitial lung disease; KL-6; lung transplant; vasculitis; myositis
Special Issues, Collections and Topics in MDPI journals
Prof. Dr. Elena Bargagli

E-Mail Website
Guest Editor
Respiratory Diseases and Lung Transplant Unit, Department of Medical and Surgical Sciences and Neurosciences, University of Siena, 53100 Siena, Italy
Interests: interstitial lung diseases; biomarkers; bronchoalveolar lavage; pathogenesis
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

It is time to commit to ensuring idiopathic interstitial pneumonia is no longer idiopathic! Besides this overwhelming aim, it is surely true that a new era for a better comprehension of these heterogeneous and potentially lethal interstitial lung diseases is approaching. The incoming implementation of omics sciences and of the principles of precision medicine is expected to revolutionize our scientific and clinical approach for our patients in order to eventually provide earlier and more accurate diagnosis, as well as targeted therapies.

The present Special Issue of IJMS is specifically dedicated to the publication of scientific works aiming to shed light on the molecular features of diffuse fibrotic parenchymal lung disorders. Submissions with an emphasis on emerging approaches are welcome. We are particularly eager to publish findings in the omics sciences (genomics, metabolomics, proteomics, lipidomics, and glycomics), cytofluorimetric assays, artificial intelligence and machine learning applications.

The principal aim of this Special issue is to explore the molecular pathways underlying the pathogenesis of fibrotic interstitial lung diseases and to investigate their potential application in clinical practice in terms of differential diagnosis and responses to treatment. Experimental studies, including in vitro and in vivo models and review articles, will all be considered for inclusion.

Dr. Paolo Cameli
Prof. Dr. Elena Bargagli
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • pulmonary fibrosis
  • biomarker
  • molecular pathways
  • omics sciences
  • personalized medicine

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Published Papers (3 papers)

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Research

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18 pages, 8537 KiB  
Article
NLRP3 Inflammasome Activates Endothelial-to-Mesenchymal Transition via Focal Adhesion Kinase Pathway in Bleomycin-Induced Pulmonary Fibrosis
by Wei-Chih Chen, Wen-Kuang Yu, Vincent Yi-Fong Su, Han-Shui Hsu and Kuang-Yao Yang
Int. J. Mol. Sci. 2023, 24(21), 15813; https://doi.org/10.3390/ijms242115813 - 31 Oct 2023
Cited by 9 | Viewed by 2268
Abstract
Idiopathic pulmonary fibrosis has poor clinical outcomes despite antifibrotic treatment. The nucleotide-binding domain leucine-rich repeat-containing receptor, pyrin domain-containing-3 (NLRP3) inflammasome and endothelial-to-mesenchymal transition (EndoMT) were shown to be involved in the pathogenesis of pulmonary fibrosis. However, the detailed mechanism is unknown. Our study [...] Read more.
Idiopathic pulmonary fibrosis has poor clinical outcomes despite antifibrotic treatment. The nucleotide-binding domain leucine-rich repeat-containing receptor, pyrin domain-containing-3 (NLRP3) inflammasome and endothelial-to-mesenchymal transition (EndoMT) were shown to be involved in the pathogenesis of pulmonary fibrosis. However, the detailed mechanism is unknown. Our study aimed to investigate the role of the NLRP3 inflammasome in the regulation of EndoMT in pulmonary fibrosis. The inhibition of the NLRP3 inflammasome via a caspase-1 inhibitor, Ac-YVAD-cmk (YVAD), was intraperitoneally administered to male C57BL/6 mice (8–12 weeks old) one hour before bleomycin intratracheal injection (1.5 U/kg). Immunohistochemical staining, Masson’s trichrome staining, enzyme-linked immunosorbent assay, immunofluorescence, and Western blotting were used to assess the activity of the NLRP3 inflammasome and EndoMT in lung samples from mice. Human pulmonary microvascular endothelial cells (HPMECs) were used as a model of EndoMT in vitro with YVAD and bleomycin stimulation. We observed the activation of the NLRP3 inflammasome and EndoMT (decreased vascular endothelial cadherin with increased alpha-smooth muscle actin and vimentin) in the lung samples after bleomycin. However, inhibition of the NLRP3 inflammasome significantly reduces EndoMT via inhibiting focal adhesion kinase (FAK). In vitro studies also confirmed these findings. In conclusion, NLRP3 inflammasome inhibition could reduce lung inflammation and fibrosis via the regulation of EndoMT by the FAK pathway. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Molecular Pathology, Diagnosis, and Therapeutic Strategies)
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Review

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19 pages, 945 KiB  
Review
Highlights on Future Treatments of IPF: Clues and Pitfalls
by Alessandro Libra, Enrico Sciacca, Giuseppe Muscato, Gianluca Sambataro, Lucia Spicuzza and Carlo Vancheri
Int. J. Mol. Sci. 2024, 25(15), 8392; https://doi.org/10.3390/ijms25158392 - 1 Aug 2024
Cited by 7 | Viewed by 3724
Abstract
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by irreversible scarring of lung tissue, leading to death. Despite recent advancements in understanding its pathophysiology, IPF remains elusive, and therapeutic options are limited and non-curative. This review aims to synthesize the latest [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by irreversible scarring of lung tissue, leading to death. Despite recent advancements in understanding its pathophysiology, IPF remains elusive, and therapeutic options are limited and non-curative. This review aims to synthesize the latest research developments, focusing on the molecular mechanisms driving the disease and on the related emerging treatments. Unfortunately, several phase 2 studies showing promising preliminary results did not meet the primary endpoints in the subsequent phase 3, underlying the complexity of the disease and the need for new integrated endpoints. IPF remains a challenging condition with a complex interplay of genetic, epigenetic, and pathophysiological factors. Ongoing research into the molecular keystones of IPF is critical for the development of targeted therapies that could potentially stop the progression of the disease. Future directions include personalized medicine approaches, artificial intelligence integration, growth in genetic insights, and novel drug targets. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Molecular Pathology, Diagnosis, and Therapeutic Strategies)
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Other

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13 pages, 3761 KiB  
Perspective
Maximizing the Therapeutic Effect of Endothelin Receptor Antagonists in Pulmonary Fibrosis: A Paradigm for Treating the Disease
by Jerome Cantor
Int. J. Mol. Sci. 2024, 25(8), 4184; https://doi.org/10.3390/ijms25084184 - 10 Apr 2024
Cited by 2 | Viewed by 1326
Abstract
Using a lipopolysaccharide model of acute lung injury, we previously showed that endothelin-1 (ET-1), a potent mediator of vasoconstriction, may act as a “gatekeeper” for the influx of inflammatory cells into the lung. These studies provided a rationale for testing the effect of [...] Read more.
Using a lipopolysaccharide model of acute lung injury, we previously showed that endothelin-1 (ET-1), a potent mediator of vasoconstriction, may act as a “gatekeeper” for the influx of inflammatory cells into the lung. These studies provided a rationale for testing the effect of HJP272, an endothelin receptor antagonist (ERA), in hamster models of pulmonary fibrosis induced by intratracheal instillation of either bleomycin (BLM) or amiodarone (AM). To determine the temporal effects of blocking ET-1 activity, animals were given HJP272 either 1 h before initiation of lung injury or 24 h afterward. The results indicated that pretreatment with this agent caused significant reductions in various inflammatory parameters, whereas post-treatment was ineffective. This finding suggests that ERAs are only effective at a very early stage of pulmonary fibrosis and explains their lack of success in clinical trials involving patients with this disease. Nevertheless, ERAs could serve as prophylactic agents when combined with drugs that may induce pulmonary fibrosis. Furthermore, developing a biomarker for the initial changes in the lung extracellular matrix could increase the efficacy of ERAs and other therapeutic agents in preventing the progression of the disease. While no such biomarker currently exists, we propose the ratio of free to peptide-bound desmosine, a unique crosslink of elastin, as a potential candidate for detecting the earliest modifications in lung microarchitecture associated with pulmonary fibrosis. Full article
(This article belongs to the Special Issue Pulmonary Fibrosis: Molecular Pathology, Diagnosis, and Therapeutic Strategies)
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