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Pulmonary fibrosis idiopathic

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https://www.readbyqxmd.com/read/28817691/rac2-is-required-for-alternative-macrophage-activation-and-bleomycin-induced-pulmonary-fibrosis-a-macrophage-autonomous-phenotype
#1
Shweta Joshi, Alok R Singh, Simon S Wong, Muamera Zulcic, Min Jiang, Annie Pardo, Moises Selman, James S Hagood, Donald L Durden
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by cellular phenotype alterations and deposition of extracellular matrix proteins. The alternative activation of macrophages in the lungs has been associated as a major factor promoting pulmonary fibrosis, however the mechanisms underlying this phenomenon are poorly understood. In the present study, we have defined a molecular mechanism by which signals transmitted from the extracellular matrix via the α4β1 integrin lead to the activation of Rac2 which regulates alternative macrophage differentiation, a signaling axis within the pulmonary macrophage compartment required for bleomycin induced pulmonary fibrosis...
2017: PloS One
https://www.readbyqxmd.com/read/28817393/increased-expiratory-computed-tomography-density-reveals-possible-abnormalities-in-radiologically-preserved-lung-parenchyma-in-idiopathic-pulmonary-fibrosis
#2
Valentina Petroulia, Manuela Funke, Pascal Zumstein, Sabina Berezowska, Lukas Ebner, Thomas Geiser, Nenad Torbica, Johannes Heverhagen, Alexander Poellinger
OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a progressive lethal chronic lung disease with unclear pathogenesis. Radiological hallmark is the pattern of usual interstitial pneumonia accentuated in peripheral and basal areas with otherwise preserved lung structure. One hypothesis is that alveolar collapse and consequent induration lead to fibrotic transformation of lung tissue. The aim of the study was to investigate normal-appearing tissue during expiration for signs of collapsibility and differences from other diseases or controls...
August 16, 2017: Investigative Radiology
https://www.readbyqxmd.com/read/28816543/mir-541-5p-regulates-lung-fibrosis-by-targeting-cyclic-nucleotide-phosphodiesterase-1a
#3
Liqin Ren, Chunyan Yang, Yongfeng Dou, Renhui Zhan, Yi Sun, Yan Yu
AIM OF THE STUDY: Idiopathic pulmonary fibrosis (IPF) is a lethal human disease with short survival time and few treatment options. In this study, we aim to demonstrate that cyclic nucleotide phosphodiesterase 1A (PDE1A), a Ca2+/calmodulin-stimulating PDE family member, plays a critical role in the induction of fibrosis and angiogenesis in the lung. MATERIALS AND METHODS: To induce pulmonary damage, adult male SD rats were treated with bleomycin in a dose of 6 mg/kg body weight by a single intratracheal instillation...
August 17, 2017: Experimental Lung Research
https://www.readbyqxmd.com/read/28814671/antifibrotic-role-of-vascular-endothelial-growth-factor-in-pulmonary-fibrosis
#4
Lynne A Murray, David M Habiel, Miriam Hohmann, Ana Camelo, Huilan Shang, Yang Zhou, Ana Lucia Coelho, Xueyan Peng, Mridu Gulati, Bruno Crestani, Matthew A Sleeman, Tomas Mustelin, Meagan W Moore, Changwan Ryu, Awo D Osafo-Addo, Jack A Elias, Chun G Lee, Buqu Hu, Jose D Herazo-Maya, Darryl A Knight, Cory M Hogaboam, Erica L Herzog
The chronic progressive decline in lung function observed in idiopathic pulmonary fibrosis (IPF) appears to result from persistent nonresolving injury to the epithelium, impaired restitution of the epithelial barrier in the lung, and enhanced fibroblast activation. Thus, understanding these key mechanisms and pathways modulating both is essential to greater understanding of IPF pathogenesis. We examined the association of VEGF with the IPF disease state and preclinical models in vivo and in vitro. Tissue and circulating levels of VEGF were significantly reduced in patients with IPF, particularly in those with a rapidly progressive phenotype, compared with healthy controls...
August 17, 2017: JCI Insight
https://www.readbyqxmd.com/read/28814429/nintedanib-macrophage-activation-and-ameliorates-vascular-and-fibrotic-manifestations-in-the-fra2-mouse-model-of-systemic-sclerosis
#5
Jingang Huang, Christiane Maier, Yun Zhang, Alina Soare, Clara Dees, Christian Beyer, Ulrike Harre, Chih-Wei Chen, Oliver Distler, Georg Schett, Lutz Wollin, Jörg H W Distler
BACKGROUND: Nintedanib is an inhibitor targeting platelet-derived growth factor receptor, fibroblast growth factor receptor and vascular endothelial growth factor receptor tyrosine kinases that has recently been approved for the treatment of idiopathic pulmonary fibrosis. The aim of this study was to analyse the effects of nintedanib in the fos-related antigen-2 (Fra2) mouse model of systemic sclerosis (SSc). METHODS: The effects of nintedanib on pulmonary arterial hypertension with proliferation of pulmonary vascular smooth muscle cells (PVSMCs) and luminal occlusion, on microvascular disease with apoptosis of microvascular endothelial cells (MVECs) and on fibroblast activation with myofibroblast differentiation and accumulation of extracellular matrix were analysed...
August 16, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28813161/circulating-mtdna-as-a-mechanism-based-prognostic-biomarker-for-idiopathic-pulmonary-fibrosis
#6
Suzanne M Cloonan
No abstract text is available yet for this article.
August 16, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28811360/involvement-of-midkine-in-the-development-of-pulmonary-fibrosis
#7
Kenichi Misa, Yoshinori Tanino, Xintao Wang, Takefumi Nikaido, Masami Kikuchi, Yuki Sato, Ryuichi Togawa, Mishie Tanino, Shinya Tanaka, Kenji Kadomatsu, Mitsuru Munakata
Midkine is a low-molecular-weight heparin-binding protein that is strongly expressed mainly in the midgestation period and has various physiological activities such as in development and cell migration. Midkine has been reported to be strongly expressed in cancer cells and in inflammation and repair processes, and to be involved in the pathogenesis of various diseases. However, its role in the lung is poorly understood. In this study, we analyzed the clinical characteristics of idiopathic pulmonary fibrosis patients in relation to midkine expression and used a mouse bleomycin-induced pulmonary fibrosis model to investigate the role of midkine in pulmonary fibrosis...
August 2017: Physiological Reports
https://www.readbyqxmd.com/read/28810065/olodaterol-shows-anti-fibrotic-efficacy-in-in-vitro-and-in-vivo-models-of-pulmonary-fibrosis
#8
Franziska Elena Herrmann, Lutz Wollin, Johannes Wirth, Florian Gantner, Bärbel Lämmle, Eva Wex
BACKGROUND AND PURPOSE: Idiopathic pulmonary fibrosis (IPF) is a fatal respiratory disease characterized by excessive fibroblast activation ultimately leading to scarring of the lungs. Although, the activation of β2 -adrenergic receptors (β2 -AR) has been shown to inhibit pro-fibrotic events primarily in cell lines, the role of β2 -AR agonism has not yet been fully characterized. The aim of our study was to explore the anti-fibrotic activity of the long-acting β2 -AR-agonist olodaterol in primary human lung fibroblasts, and in murine models of pulmonary fibrosis...
August 15, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28809513/vascular-endothelial-growth-factor-in-idiopathic-pulmonary-fibrosis-an-imbalancing-act
#9
Sergei P Atamas
No abstract text is available yet for this article.
August 15, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28804709/exploring-animal-models-that-resemble-idiopathic-pulmonary-fibrosis
#10
REVIEW
Jun Tashiro, Gustavo A Rubio, Andrew H Limper, Kurt Williams, Sharon J Elliot, Ioanna Ninou, Vassilis Aidinis, Argyrios Tzouvelekis, Marilyn K Glassberg
Large multicenter clinical trials have led to two recently approved drugs for patients with idiopathic pulmonary fibrosis (IPF); yet, both of these therapies only slow disease progression and do not provide a definitive cure. Traditionally, preclinical trials have utilized mouse models of bleomycin (BLM)-induced pulmonary fibrosis-though several limitations prevent direct translation to human IPF. Spontaneous pulmonary fibrosis occurs in other animal species, including dogs, horses, donkeys, and cats. While the fibrotic lungs of these animals share many characteristics with lungs of patients with IPF, current veterinary classifications of fibrotic lung disease are not entirely equivalent...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28804700/abridged-version-of-the-awmf-guideline-for-the-medical-clinical-diagnostics-of-indoor-mould-exposure-s2k-guideline-of-the-german-society-of-hygiene-environmental-medicine-and-preventive-medicine-ghup-in-collaboration-with-the-german-association-of-allergists
#11
Gerhard A Wiesmüller, Birger Heinzow, Ute Aurbach, Karl-Christian Bergmann, Albrecht Bufe, Walter Buzina, Oliver A Cornely, Steffen Engelhart, Guido Fischer, Thomas Gabrio, Werner Heinz, Caroline E W Herr, Jörg Kleine-Tebbe, Ludger Klimek, Martin Köberle, Herbert Lichtnecker, Thomas Lob-Corzilius, Rolf Merget, Norbert Mülleneisen, Dennis Nowak, Uta Rabe, Monika Raulf, Hans Peter Seidl, Jens-Oliver Steiß, Regine Szewszyk, Peter Thomas, Kerttu Valtanen, Julia Hurraß
This article is an abridged version of the AWMF mould guideline "Medical clinical diagnostics of indoor mould exposure" presented in April 2016 by the German Society of Hygiene, Environmental Medicine and Preventive Medicine (Gesellschaft für Hygiene, Umweltmedizin und Präventivmedizin, GHUP), in collaboration with the above-mentioned scientific medical societies, German and Austrian societies, medical associations and experts. Indoor mould growth is a potential health risk, even if a quantitative and/or causal relationship between the occurrence of individual mould species and health problems has yet to be established...
2017: Allergo Journal International
https://www.readbyqxmd.com/read/28802694/exposure-to-ambient-particulate-matter-is-associated-with-accelerated-functional-decline-in-idiopathic-pulmonary-fibrosis
#12
Christopher J Winterbottom, Rupal J Shah, Karen C Patterson, Maryl E Kreider, Reynold A Panettieri, Belinda Rivera-Lebron, Wallace T Miller, Leslie A Litzky, Trevor M Penning, Krista Heinlen, Tara Jackson, A Russell Localio, Jason D Christie
BACKGROUND: Idiopathic pulmonary fibrosis (IPF), a progressive disease with unknown pathogenesis, may in part be due to an abnormal response to injurious stimuli by alveolar epithelial cells. Air pollution and particulate matter inhalation evoke a wide variety of pulmonary and systemic inflammatory diseases. We therefore hypothesized that increased average ambient particulate matter (PM) concentrations would be associated with an accelerated rate of decline in forced vital capacity (FVC) in IPF...
August 9, 2017: Chest
https://www.readbyqxmd.com/read/28802277/microbiome-in-lung-explants-of-idiopathic-pulmonary-fibrosis-a-case-control-study-in-patients-with-end-stage-fibrosis
#13
Georgios D Kitsios, Mauricio Rojas, Daniel J Kass, Adam Fitch, John C Sembrat, Shulin Qin, Kristen L Veraldi, Kevin F Gibson, Kathleen Lindell, Joseph M Pilewski, Barbara Methe, Kelvin Li, John McDyer, Bryan J McVerry, Alison Morris
The microbiome has been proposed to play a role in the progression of idiopathic pulmonary fibrosis (IPF) based on bronchoalveolar lavage analyses, but the microbiome of lung tissue in IPF has not been explored. In a case-control study of lung explants analysed by 16S rRNA gene sequencing, we could not reliably detect bacterial DNA in basilar tissue samples from patients with either chronic or acute exacerbations of IPF, in contrast to control candidate-donor lungs or cystic fibrosis explants. Thus, our data do not indicate microbiome alterations in regions of IPF lung with advanced fibrosis...
August 11, 2017: Thorax
https://www.readbyqxmd.com/read/28800589/prognostic-factors-and-outcomes-in-japanese-lung-transplant-candidates-with-interstitial-lung-disease
#14
Kohei Ikezoe, Tomohiro Handa, Kiminobu Tanizawa, Toyofumi F Chen-Yoshikawa, Takeshi Kubo, Akihiro Aoyama, Hideki Motoyama, Kyoko Hijiya, Shinsaku Tokuda, Yoshinari Nakatsuka, Yuko Yamamoto, Ayako Oshima, Shin-Ichi Harashima, Sonoko Nagai, Toyohiro Hirai, Hiroshi Date, Kazuo Chin
OBJECTIVE: Young patients with advanced interstitial lung disease (ILD) are potential candidates for cadaveric lung transplantation. This study aimed to examine clinical features, outcomes, and prognostic factors in Japanese ILD patients awaiting lung transplantation. METHODS: We investigated the clinical features and outcomes of 77 consecutive candidates with ILD who were referred to Kyoto University Hospital and subsequently actively listed for lung transplant in the Japan Organ Transplant Network between 2010 and 2014...
2017: PloS One
https://www.readbyqxmd.com/read/28799781/microrna-29c-prevents-pulmonary-fibrosis-by-regulating-epithelial-cell-renewal-and-apoptosis
#15
Ting Xie, Jiurong Liang, Yan Geng, Ningshan Liu, Adrianne Kurkciyan, Vrishika Kulur, Dong Leng, Nan Deng, Zhenqiu Liu, Jianbo Song, Peter Chen, Paul W Noble, Dianhua Jiang
Successful repair and renewal of alveolar epithelial cells are critical in prohibiting the accumulation of myofibroblasts in pulmonary fibrogenesis. MicroRNAs (miRNAs) are multi-focal regulators involved in lung injury and repair. But the contribution of miRNAs to AEC2 renewal and apoptosis is incompletely understood. We report that microRNA-29c (MiR-29c) expression is lower in AEC2s of individuals with idiopathic pulmonary fibrosis (IPF) than healthy lungs. Epithelial cells overexpressing miR-29c show higher proliferative rate and viability...
August 11, 2017: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/28798214/role-of-atmospheric-pollution-on-the-natural-history-of-idiopathic-pulmonary-fibrosis
#16
Lucile Sesé, Hilario Nunes, Vincent Cottin, Shreosi Sanyal, Morgane Didier, Zohra Carton, Dominique Israel-Biet, Bruno Crestani, Jacques Cadranel, Benoit Wallaert, Abdellatif Tazi, Bernard Maître, Grégoire Prévot, Sylvain Marchand-Adam, Stéphanie Guillot-Dudoret, Annelyse Nardi, Sandra Dury, Violaine Giraud, Anne Gondouin, Karine Juvin, Raphael Borie, Marie Wislez, Dominique Valeyre, Isabella Annesi-Maesano
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) has an unpredictable course corresponding to various profiles: stability, physiological disease progression and rapid decline. A minority of patients experience acute exacerbations (AEs). A recent study suggested that ozone and nitrogen dioxide might contribute to the occurrence of AE. We hypothesised that outdoor air pollution might influence the natural history of IPF. METHODS: Patients were selected from the French cohort COhorte FIbrose (COFI), a national multicentre longitudinal prospective cohort of IPF (n=192)...
August 10, 2017: Thorax
https://www.readbyqxmd.com/read/28797401/a-58-year-old-man-with-position-dependent-nocturnal-dyspnea
#17
Anke Schertel, Christian M Horvath, Jacqueline Pichler Hefti, John-David Aubert, Anne-Kathrin Brill
A 58-year-old man with idiopathic pulmonary fibrosis, who had received a right-sided single-lung transplant 2 years earlier, was referred to the sleep clinic for the assessment of nocturnal position-dependent episodes of dyspnea and frequent arousals when lying on his right side. There was no subjective worsening of daytime respiratory symptoms, but he complained of fatigue and unrefreshing sleep. His Epworth Sleepiness Scale score was 12/24. After lung transplantation he had a favorable course while receiving immunosuppression with prednisolone, everolimus, and mycophenolate mofetil...
August 2017: Chest
https://www.readbyqxmd.com/read/28796717/utility-of-flexible-bronchoscopic-cryobiopsy-for-diagnosis-of-diffuse-parenchymal-lung-diseases
#18
Robert J Lentz, Trevor M Taylor, Jonathan A Kropski, Kim L Sandler, Joyce E Johnson, Timothy S Blackwell, Fabien Maldonado, Otis B Rickman
BACKGROUND: Initial reports of transbronchial cryobiopsy for diffuse parenchymal lung disease (DPLD) suggest the diagnostic yield approaches that of surgical lung biopsy (SLB) with an excellent safety profile. Centers performing cryobiopsy differ significantly in procedure technique; an optimal technique minimizing complications but still capable of diagnosing a wide range of DPLDs has not been established. We evaluated our practice of flexible bronchoscopic cryobiopsy in a primarily outpatient setting for patients who required a tissue diagnosis for DPLD of uncertain etiology...
August 7, 2017: Journal of Bronchology & Interventional Pulmonology
https://www.readbyqxmd.com/read/28793886/cathepsin-s-degraded-decorin-are-elevated-in-fibrotic-lung-disorders-development-and-biological-validation-of-a-new-serum-biomarker
#19
S N Kehlet, C L Bager, N Willumsen, B Dasgupta, C Brodmerkel, M Curran, S Brix, D J Leeming, M A Karsdal
BACKGROUND: Decorin is one of the most abundant proteoglycans of the extracellular matrix and is mainly secreted and deposited in the interstitial matrix by fibroblasts where it plays an important role in collagen turnover and tissue homeostasis. Degradation of decorin might disturb normal tissue homeostasis contributing to extracellular matrix remodeling diseases. Here, we present the development and validation of a competitive enzyme-linked immunosorbent assay (ELISA) quantifying a specific fragment of degraded decorin, which has potential as a novel non-invasive serum biomarker for fibrotic lung disorders...
August 9, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28783377/extracellular-mitochondrial-dna-is-generated-by-fibroblasts-and-predicts-death-in-idiopathic-pulmonary-fibrosis
#20
Changwan Ryu, Huanxing Sun, Mridu Gulati, Jose D Herazo-Maya, Yonglin Chen, Awo Osafo-Addo, Caitlin Brandsdorfer, Julia Winkler, Christina Blaul, Jaden Faunce, Hongyi Pan, Tony Woolard, Argyrios Tzouvelekis, Danielle E Antin-Ozerkis, Jonathan T Puchalski, Martin Slade, Anjelica L Gonzalez, Daniel F Bogenhagen, Varvara Kirillov, Carol Feghali-Bostwick, Kevin Gibson, Kathleen Lindell, Raimund I Herzog, Charles S Dela Cruz, Wajahat Mehal, Naftali Kaminski, Erica Herzog, Glenda Trujillo
RATIONALE: Idiopathic pulmonary fibrosis (IPF) involves the accumulation of alpha smooth muscle actin (αSMA) expressing myofibroblasts arising from interactions with soluble mediators such as transforming growth factor beta-1 (TGFβ1), and mechanical influences such as local tissue stiffness. While IPF fibroblasts are enriched for aerobic glycolysis and innate immune receptor activation, innate immune ligands related to mitochondrial injury, such as extracellular mitochondrial DNA (mtDNA) have not been identified in IPF...
August 7, 2017: American Journal of Respiratory and Critical Care Medicine
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