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Special Issue "The New Frontier in Pulmonary Fibrosis"

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Pulmonology".

Deadline for manuscript submissions: 31 August 2019

Special Issue Editor

Guest Editor
Dr. Andrew J. Bryant

University of Florida College of Medicine
Website | E-Mail
Interests: idiopathic pulmonary fibrosis (IPF); pulmonary hypertension; myeloid-derived suppressor cells (MDSC); vascular remodeling; hypoxia; bleomycin-induced pulmonary fibrosis; interstitial lung disease (ILD)

Special Issue Information

Dear Colleagues,

As you are aware, idiopathic pulmonary fibrosis (IPF) is an unrelenting and incurable disease characterized primarily by progressive stiffening and scarring of the lungs. In IPF, alveolar epithelial cell dysfunction and aberrant proliferation of activated fibroblasts—with contemporaneous vascular endothelial cell dysfunction—contribute to the development of alveolar thickening, which then leads to the formation of fibroblastic foci and the honeycombing of the lungs and, ultimately, respiratory failure and death.  This Special Issue focuses on novel mechanisms, diagnostic techniques, and therapeutics contributing to IPF, including aberrant metabolic and immunologic responses to lung tissue regeneration, as well as frequent comorbidities seen in IPF such as pulmonary hypertension. We warmly welcome a broad assortment of submissions, including original papers and reviews, on the forefront of this stimulating topic.

Dr. Andrew J. Bryant
Guest Editor

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 papers will be 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. Journal of Clinical Medicine is an international peer-reviewed open access monthly 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 1800 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

  • Pulmonary fibrosis
  • Fibroblast
  • Extracellular matrix
  • Vascular endothelium
  • Hypoxia
  • Alveolar epithelial cell
  • Lung immunology
  • Immunotherapy
  • Metabolism
  • Respiratory physiology

Published Papers (3 papers)

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Research

Open AccessArticle
Exhalative Breath Markers Do Not Offer for Diagnosis of Interstitial Lung Diseases: Data from the European IPF Registry (eurIPFreg) and Biobank
J. Clin. Med. 2019, 8(5), 643; https://doi.org/10.3390/jcm8050643
Received: 5 April 2019 / Revised: 26 April 2019 / Accepted: 4 May 2019 / Published: 9 May 2019
PDF Full-text (1111 KB)
Abstract
Background: New biomarkers are urgently needed to facilitate diagnosis in Interstitial Lung Diseases (ILD), thus reducing the need for invasive procedures, and to enable tailoring and monitoring of medical treatment. Methods: In this study we investigated if patients with idiopathic pulmonary fibrosis [...] Read more.
Background: New biomarkers are urgently needed to facilitate diagnosis in Interstitial Lung Diseases (ILD), thus reducing the need for invasive procedures, and to enable tailoring and monitoring of medical treatment. Methods: In this study we investigated if patients with idiopathic pulmonary fibrosis (IPF; n = 21), non-IPF ILDs (n = 57) and other lung diseases (chronic obstructive pulmonary disease (COPD) n = 24, lung cancer (LC) n = 16) as well as healthy subjects (n = 20) show relevant differences in exhaled NO (FeNO; Niox MINO), or in eicosanoid (PGE2, 8-isoprostane; enzyme-linked immunosorbent assay (ELISA)) levels as measured in exhaled breath condensates (EBC) and bronchoalveolar lavage fluids (BALF). Results: There was no significant difference in FeNO values between IPF, non-IPF ILDs and healthy subjects, although some individual patients showed highly elevated FeNO. On the basis of the FeNO signal, it was neither possible to differentiate between the kind of disease nor to detect exacerbations. In addition, there was no correlation between FeNO values and lung function. The investigation of the eicosanoids in EBCs was challenging (PGE2) or unreliable (8-isoprostane), but worked out well in BALF. A significant increase of free 8-isoprostane was observed in BALF, but not in EBCs, of patients with IPF, hypersensitivity pneumonitis (HP) and sarcoidosis, possibly indicating severity of oxidative stress. Conclusions: FeNO-measurements are not of diagnostic benefit in different ILDs including IPF. The same holds true for PGE2 and 8-isoprostane in EBC by ELISA. Full article
(This article belongs to the Special Issue The New Frontier in Pulmonary Fibrosis)
Open AccessArticle
High-Resolution Computed Tomography (HRCT) Reflects Disease Progression in Patients with Idiopathic Pulmonary Fibrosis (IPF): Relationship with Lung Pathology
J. Clin. Med. 2019, 8(3), 399; https://doi.org/10.3390/jcm8030399
Received: 3 February 2019 / Revised: 12 March 2019 / Accepted: 18 March 2019 / Published: 22 March 2019
PDF Full-text (1821 KB) | HTML Full-text | XML Full-text | Supplementary Files
Abstract
High-Resolution Computed Tomography (HRCT) plays a central role in diagnosing Idiopathic Pulmonary Fibrosis (IPF) while its role in monitoring disease progression is not clearly defined. Given the variable clinical course of the disease, we evaluated whether HRCT abnormalities predict disease behavior and correlate [...] Read more.
High-Resolution Computed Tomography (HRCT) plays a central role in diagnosing Idiopathic Pulmonary Fibrosis (IPF) while its role in monitoring disease progression is not clearly defined. Given the variable clinical course of the disease, we evaluated whether HRCT abnormalities predict disease behavior and correlate with functional decline in untreated IPF patients. Forty-nine patients (with HRCT1) were functionally categorized as rapid or slow progressors. Twenty-one had a second HRCT2. Thirteen patients underwent lung transplantation and pathology was quantified. HRCT Alveolar (AS) and Interstitial Scores (IS) were assessed and correlated with Forced Vital Capacity (FVC) decline between HRCT1 and HRCT2. At baseline, AS was greater in rapids than in slows, while IS was similar in the two groups. In the 21 subjects with HRCT2, IS increased over time in both slows and rapids, while AS increased only in rapids. The IS change from HRCT1 to HRCT2 normalized per month correlated with FVC decline/month in the whole population, but the change in AS did not. In the 13 patients with pathology, the number of total lymphocytes was higher in rapids than in slows and correlated with AS. Quantitative estimation of HRCTs AS and IS reflects the distinct clinical and pathological behavior of slow and rapid decliners. Furthermore, AS, which reflects the immune/inflammatory infiltrate in lung tissue, could be a useful tool to differentiate rapid from slow progressors at presentation. Full article
(This article belongs to the Special Issue The New Frontier in Pulmonary Fibrosis)
Figures

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Open AccessArticle
Inhibition of NADPH Oxidase 4 (NOX4) Signaling Attenuates Tuberculous Pleural Fibrosis
J. Clin. Med. 2019, 8(1), 116; https://doi.org/10.3390/jcm8010116
Received: 21 November 2018 / Revised: 15 January 2019 / Accepted: 15 January 2019 / Published: 18 January 2019
PDF Full-text (10704 KB) | HTML Full-text | XML Full-text | Supplementary Files
Abstract
Nicotinamide adenine dinucleotide phosphate (NADPH) oxidase [NOX] enzymes serve several hemostatic and host defense functions in various lung diseases, but the role of NOX4 signaling in tuberculous pleurisy is not well understood. The role of NOX4 signaling in tuberculous pleural fibrosis was studied [...] Read more.
Nicotinamide adenine dinucleotide phosphate (NADPH) oxidase [NOX] enzymes serve several hemostatic and host defense functions in various lung diseases, but the role of NOX4 signaling in tuberculous pleurisy is not well understood. The role of NOX4 signaling in tuberculous pleural fibrosis was studied using invitro pleural mesothelial cell (PMC) experiments and a murine model of Mycobacterium bovis bacillus Calmette–Guérin (BCG) pleural infection. The production of NOX4 reactive oxygen species (NOX4–ROS) and the epithelial mesenchymal transition (EMT) in PMCs were both induced by heat-killed mycobacterium tuberculosis (HKMT). In cultured PMCs, HKMT-induced collagen-1 synthesis and EMT were blocked by pretreatment with small interfering RNA (siRNA) NOX4. Moreover, NOX4–ROS production and subsequent fibrosis were reduced by treatment with losartan and the toll-like receptor 4 (TLR4) inhibitor TAK-242. The HKMT-induced EMT and intracellular ROS production were mediated by NOX4 via the activation of extracellular signal-regulated kinase (ERK) signaling. Finally, in a BCG-induced pleurisy model, recruitment of inflammatory pleural cells, release of inflammatory cytokines, and thickened mesothelial fibrosis were attenuated by SiNOX4 compared to SiCon. Our study identified that HKMT-induced pleural fibrosis is mediated by NOX4–ERK–ROS via TLR4 and Angiotensin II receptor type1 (AT1R). There results suggest that NOX4 may be a novel therapeutic target for intervention in tuberculous pleural fibrosis. Full article
(This article belongs to the Special Issue The New Frontier in Pulmonary Fibrosis)
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Figure 1

J. Clin. Med. EISSN 2077-0383 Published by MDPI AG, Basel, Switzerland RSS E-Mail Table of Contents Alert
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