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Cells
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State of the Art in Idiopathic Pulmonary Fibrosis-Series 2
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State of the Art in Idiopathic Pulmonary Fibrosis-Series 2

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cellular Pathology".

Deadline for manuscript submissions: closed (15 December 2023) | Viewed by 7536

Special Issue Editors

Prof. Dr. Malgorzata Wygrecka

E-Mail Website
Guest Editor
Center for Infection and Genomics of The Lung, Justus-Liebig University Giessen, Aulweg 132, 35392 Giessen, Germany
Interests: lung fibrosis; ECM; coagulation factors; glycosaminoglycans
Special Issues, Collections and Topics in MDPI journals
Prof. Dr. Elie El Agha

E-Mail Website
Guest Editor
Institute for Lung Health (ILH), Justus-Liebig University Giessen, Aulweg 132, 35392 Giessen, Germany
Interests: lung development; stem cells; mesenchymal cells; tissue injury and repair
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Idiopathic pulmonary fibrosis (IPF) is a lethal disease of unknown etiology, elusive pathogenesis, and very limited therapeutic options. The onset and progression of IPF are influenced by multiple environmental and intrinsic factors, such as exposure to harmful substances, aging and genetic predisposition; however, the magnitude of the contribution of these factors to IPF and the chronological order of downstream pathogenic events remain uncertain. The main hallmarks of IPF are the abnormal activation of lung epithelial cells and the accumulation of fibroblasts/myofibroblasts along with the excessive deposition of extracellular matrix proteins. The aforementioned processes eventually lead to irreversible alveolar scarring, organ malfunction, and death. The incidence and prevalence of IPF are increasing at an alarming rate with the aging population. Recent technological advances and interdisciplinary approaches unmasked the involvement of a broad spectrum of molecular and cellular mediators in the pathogenesis of IPF. Molecules as diverse as lipids, RNAs, or peptides, along with a plethora of inflammatory, epithelial, and mesenchymal cell subpopulations turned out to drive maladaptive remodeling to lung tissue. The multifactorial nature of IPF and the lack of robust translational models represent an enormous challenge for the development of successful therapeutic approaches. By critically evaluating the complexity of the disease and the translational value of pre-clinical studies, we would like to provide here a platform for conceptual and technological innovation in the field of IPF and shed light on new therapeutic strategies that may become a part of future treatment options.

This Special Issue encourages the submission of original research articles, reviews or methodological articles dealing with various aspects of IPF research including, but not limited to, the use of in vivo, ex vivo or in vitro models to study lung fibrosis, as well as novel molecular or cellular mediators of lung tissue injury and repair. Studies with clear translational potential are especially welcome.

Prof. Dr. Malgorzata Wygrecka
Prof. Dr. Elie El Agha
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. Cells is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). 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

  • IPF
  • ECM
  • translational models of lung fibrosis
  • cellular and molecular mediators

Published Papers (3 papers)

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Research

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18 pages, 3890 KiB  
Article
Mitochondrial Dysfunction in Lung Resident Mesenchymal Stem Cells from Idiopathic Pulmonary Fibrosis Patients
by Josep Mercader-Barceló, Aina Martín-Medina, Joan Truyols-Vives, Gabriel Escarrer-Garau, Linda Elowsson, Ana Montes-Worboys, Carlos Río-Bocos, Josep Muncunill-Farreny, Julio Velasco-Roca, Anna Cederberg, Måns Kadefors, Maria Molina-Molina, Gunilla Westergren-Thorsson and Ernest Sala-Llinàs
Cells 2023, 12(16), 2084; https://doi.org/10.3390/cells12162084 - 17 Aug 2023
Cited by 3 | Viewed by 1363
Abstract
Idiopathic pulmonary fibrosis (IPF) is characterized by an aberrant repair response with uncontrolled turnover of extracellular matrix involving mesenchymal cell phenotypes, where lung resident mesenchymal stem cells (LRMSC) have been supposed to have an important role. However, the contribution of LRMSC in lung [...] Read more.
Idiopathic pulmonary fibrosis (IPF) is characterized by an aberrant repair response with uncontrolled turnover of extracellular matrix involving mesenchymal cell phenotypes, where lung resident mesenchymal stem cells (LRMSC) have been supposed to have an important role. However, the contribution of LRMSC in lung fibrosis is not fully understood, and the role of LRMSC in IPF remains to be elucidated. Here, we performed transcriptomic and functional analyses on LRMSC isolated from IPF and control patients (CON). Both over-representation and gene set enrichment analyses indicated that oxidative phosphorylation is the major dysregulated pathway in IPF LRMSC. The most relevant differences in biological processes included complement activation, mesenchyme development, and aerobic electron transport chain. Compared to CON LRMSC, IPF cells displayed impaired mitochondrial respiration, lower expression of genes involved in mitochondrial dynamics, and dysmorphic mitochondria. These changes were linked to an impaired autophagic response and a lower mRNA expression of pro-apoptotic genes. In addition, IPF TGFβ-exposed LRMSC presented different expression profiles of mitochondrial-related genes compared to CON TGFβ-treated cells, suggesting that TGFβ reinforces mitochondrial dysfunction. In conclusion, these results suggest that mitochondrial dysfunction is a major event in LRMSC and that their occurrence might limit LRMSC function, thereby contributing to IPF development. Full article
(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis-Series 2)
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17 pages, 9098 KiB  
Article
A New Model of Acute Exacerbation of Experimental Pulmonary Fibrosis in Mice
by Céline-Hivda Yegen, Liasmine Haine, Kevin Da Costa Ferreira, Dominique Marchant, Jean-Francois Bernaudin, Carole Planès, Nicolas Voituron and Emilie Boncoeur
Cells 2022, 11(21), 3379; https://doi.org/10.3390/cells11213379 - 26 Oct 2022
Cited by 5 | Viewed by 2293
Abstract
Rationale: idiopathic pulmonary fibrosis (IPF) is the most severe form of fibrosing interstitial lung disease, characterized by progressive respiratory failure leading to death. IPF’s natural history is heterogeneous, and its progression unpredictable. Most patients develop a progressive decline of respiratory function over years; [...] Read more.
Rationale: idiopathic pulmonary fibrosis (IPF) is the most severe form of fibrosing interstitial lung disease, characterized by progressive respiratory failure leading to death. IPF’s natural history is heterogeneous, and its progression unpredictable. Most patients develop a progressive decline of respiratory function over years; some remain stable, but others present a fast-respiratory deterioration without identifiable cause, classified as acute exacerbation (AE). Objectives: to develop and characterize an experimental mice model of lung fibrosis AE, mimicking IPF-AE at the functional, histopathological, cellular and molecular levels. Methods: we established in C57BL/6 male mice a chronic pulmonary fibrosis using a repetitive low-dose bleomycin (BLM) intratracheal (IT) instillation regimen (four instillations of BLM every 2 weeks), followed by two IT instillations of a simple or double-dose BLM challenge to induce AE. Clinical follow-up and histological and molecular analyses were done for fibrotic and inflammatory lung remodeling analysis. Measurements and main results: as compared with a low-dose BLM regimen, this AE model induced a late burst of animal mortality, worsened lung fibrosis and remodeling, and superadded histopathological features as observed in humans IPF-AE. This was associated with stronger inflammation, increased macrophage infiltration of lung tissue and increased levels of pro-inflammatory cytokines in lung homogenates. Finally, it induced in the remodeled lung a diffuse expression of hypoxia-inducible factor 1α, a hallmark of tissular hypoxia response and a major player in the progression of IPF. Conclusion: this new model is a promising model of AE in chronic pulmonary fibrosis that could be relevant to mimic IPF-AE in preclinical trials. Full article
(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis-Series 2)
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Review

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27 pages, 3574 KiB  
Review
Combined Pulmonary Fibrosis and Emphysema: When Scylla and Charybdis Ally
by Marija Gredic, Srikanth Karnati, Clemens Ruppert, Andreas Guenther, Sergey N. Avdeev and Djuro Kosanovic
Cells 2023, 12(9), 1278; https://doi.org/10.3390/cells12091278 - 28 Apr 2023
Cited by 1 | Viewed by 3380
Abstract
Combined pulmonary fibrosis and emphysema (CPFE) is a recently recognized syndrome that, as its name indicates, involves the existence of both interstitial lung fibrosis and emphysema in one individual, and is often accompanied by pulmonary hypertension. This debilitating, progressive condition is most often [...] Read more.
Combined pulmonary fibrosis and emphysema (CPFE) is a recently recognized syndrome that, as its name indicates, involves the existence of both interstitial lung fibrosis and emphysema in one individual, and is often accompanied by pulmonary hypertension. This debilitating, progressive condition is most often encountered in males with an extensive smoking history, and is presented by dyspnea, preserved lung volumes, and contrastingly impaired gas exchange capacity. The diagnosis of the disease is based on computed tomography imaging, demonstrating the coexistence of emphysema and interstitial fibrosis in the lungs, which might be of various types and extents, in different areas of the lung and several relative positions to each other. CPFE bears high mortality and to date, specific and efficient treatment options do not exist. In this review, we will summarize current knowledge about the clinical attributes and manifestations of CPFE. Moreover, we will focus on pathophysiological and pathohistological lung phenomena and suspected etiological factors of this disease. Finally, since there is a paucity of preclinical research performed for this particular lung pathology, we will review existing animal studies and provide suggestions for the development of additional in vivo models of CPFE syndrome. Full article
(This article belongs to the Special Issue State of the Art in Idiopathic Pulmonary Fibrosis-Series 2)
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