11,261 results match your criteria Pulmonary Fibrosis Idiopathic


Endothelial to mesenchymal transition (EndMT) and vascular remodelling in pulmonary hypertension and idiopathic pulmonary fibrosis.

Expert Rev Respir Med 2020 Jul 13. Epub 2020 Jul 13.

Respiratory Translational Research Group, Department of Laboratory Medicine, School of Health Sciences, College of Health and Medicine, University of Tasmania , Launceston, Tasmania, Australia, 7248.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and irreversible fibrotic disease associated with respiratory failure. The disease remains idiopathic, but repeated alveolar epithelium injury, disruption of alveolar-capillary integrity, abnormal vascular repair, and pulmonary vascular remodelling are considered possible pathogenic mechanisms. Also, the development of comorbidities such as pulmonary hypertension (PH) could further impact disease outcome, quality of life and survival rates in IPF. Read More

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http://dx.doi.org/10.1080/17476348.2020.1795832DOI Listing

Serum latent transforming growth factor-β binding protein 4 as a novel biomarker for idiopathic pleuroparenchymal fibroelastosis.

Respir Med 2020 Jul 3;171:106077. Epub 2020 Jul 3.

Department of Respiratory Medicine, Fukuoka University Chikushi Hospital, Fukuoka, Japan. Electronic address:

Background: Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a rare idiopathic interstitial pneumonia characterized by an upper lobe-dominant interstitial increase in predominantly elastic fibers. The accumulation of cases has resulted in a refinement of the disease concept, but there are no blood biomarkers to aid in the diagnosis or prediction of a progressive phenotype among PPFE patients. Several organizers, including latent transforming growth factor-β binding protein 4 (LTBP-4), are known to be involved in elastogenesis. Read More

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http://dx.doi.org/10.1016/j.rmed.2020.106077DOI Listing

Multi-channel lung sound classification with convolutional recurrent neural networks.

Comput Biol Med 2020 Jul 23;122:103831. Epub 2020 May 23.

Signal Processing and Speech Communication Laboratory, Graz University of Technology, Graz, Austria.

In this paper, we present an approach for multi-channel lung sound classification, exploiting spectral, temporal and spatial information. In particular, we propose a frame-wise classification framework to process full breathing cycles of multi-channel lung sound recordings with a convolutional recurrent neural network. With our recently developed 16-channel lung sound recording device, we collect lung sound recordings from lung-healthy subjects and patients with idiopathic pulmonary fibrosis (IPF), within a clinical trial. Read More

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http://dx.doi.org/10.1016/j.compbiomed.2020.103831DOI Listing

Progressive fibrosing interstitial lung disease: treatable traits and therapeutic strategies.

Curr Opin Pulm Med 2020 Jul 9. Epub 2020 Jul 9.

Firestone Institute for Respiratory Health, Research Institute at St Joseph's Healthcare, Department of Medicine, McMaster University, Hamilton, Ontario, Canada.

Purpose Of Review: In this review, the authors describe therapeutic strategies for a disease group called progressive fibrosing interstitial lung disease (PF-ILD) and highlight the importance of the definition of progression, prognosis, and treatment response.

Recent Findings: Although it is a relatively new concept, the term PF-ILD has been increasingly applied in clinical research and practice. Three domains commonly used to detect the disease progression include clinical symptoms, rate of forced vital capacity (FVC) decline and the extent of fibrosis on imaging. Read More

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http://dx.doi.org/10.1097/MCP.0000000000000712DOI Listing

Fibroblast-specific IL11 signaling drives chronic inflammation in murine fibrotic lung disease.

FASEB J 2020 Jul 12. Epub 2020 Jul 12.

National Heart Research Institute Singapore, National Heart Centre Singapore, Singapore, Singapore.

Repetitive pulmonary injury causes fibrosis and inflammation that underlies chronic lung diseases such as idiopathic pulmonary fibrosis (IPF). Interleukin 11 (IL11) is important for pulmonary fibroblast activation but the contribution of fibroblast-specific IL11 activity to lung fibro-inflammation is not known. To address this gap in knowledge, we generated mice with loxP-flanked Il11ra1 and deleted the IL11 receptor in adult fibroblasts (CKO mice). Read More

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http://dx.doi.org/10.1096/fj.202001045RRDOI Listing

Additive Protective Effects of Sacubitril/Valsartan and Bosentan on Vascular Remodeling in Experimental Pulmonary Hypertension.

Cardiovasc Res 2020 Jul 11. Epub 2020 Jul 11.

INSERM UMR_S 999 «Pulmonary Hypertension: Pathophysiology and Novel Therapies», Hôpital Marie Lannelongue, 92350 Le Plessis-Robinson, France.

Aims: Although right ventricular (RV) function is an important determinant of morbidity and mortality in patients with pulmonary arterial hypertension (PAH), there is no treatment targeting directly the RV. We therefore evaluate the efficacy of sacubitril/valsartan (LCZ 696) as add-on therapy to bosentan in rats with severe pulmonary hypertension (PH).

Methods And Results: Combination therapy of LCZ 696 and bosentan has additive vascular protective effects against the pulmonary vascular remodeling and PH in two preclinical models of severe PH. Read More

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http://dx.doi.org/10.1093/cvr/cvaa200DOI Listing

Unilateral pattern of macular dystrophy and associated systemic pathology.

Arch Soc Esp Oftalmol 2020 Jul 9. Epub 2020 Jul 9.

Servicio de Oftalmología, Hospital Clínico Universitario, Valladolid, España. Electronic address:

Retinal pattern dystrophies are a heterogeneous group of generally bilateral and symmetrical maculopathies that, curiously, can be associated with different systemic diseases. This article describes a patient with unilateral pattern dystrophies, as well as associated McArdle disease and idiopathic pulmonary fibrosis. Read More

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http://dx.doi.org/10.1016/j.oftal.2020.05.040DOI Listing

Treatment patterns, healthcare resource utilization, and costs among patients with idiopathic pulmonary fibrosis treated with antifibrotic medications in US-based commercial and Medicare Supplemental claims databases: a retrospective cohort study.

BMC Pulm Med 2020 Jul 11;20(1):188. Epub 2020 Jul 11.

IBM Watson Health, 75 Binney Street, Cambridge, MA, 02142, USA.

Background: Pirfenidone and nintedanib are antifibrotic therapies which slow disease progression in idiopathic pulmonary fibrosis (IPF), an irreversible, progressive lung disease with poor prognosis. We compared adherence, persistence, and healthcare costs between patients initiating one of the two therapies.

Methods: We used the IBM Watson Health Commercial and Medicare Supplemental claims databases to select patients with IPF with ≥1 pharmacy claim for pirfenidone or nintedanib between 10/1/2014 and 6/30/2018. Read More

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http://dx.doi.org/10.1186/s12890-020-01224-5DOI Listing

Patients With Fibrotic Interstitial Lung Disease Receive Supportive and Palliative Care Just Prior to Death.

Am J Hosp Palliat Care 2020 Jul 10:1049909120938629. Epub 2020 Jul 10.

Department of Respiratory and Sleep Medicine, Austin Health, Heidelberg, Victoria, Australia.

Background: Fibrotic interstitial lung diseases (f-ILDs) are often progressive and incurable. As patients experience significant symptoms and have a poor prognosis, early palliative care referral is recommended.

Objective: To examine the care delivered to patients with f-ILD during the terminal hospital admission and the past 2 years of life. Read More

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http://dx.doi.org/10.1177/1049909120938629DOI Listing

Diagnosing idiopathic pulmonary fibrosis: Has the time for surgical lung biopsy passed?

Respirology 2020 Jul 9. Epub 2020 Jul 9.

Interstitial Lung Diseases Unit, Department of Respiratory and Sleep Medicine, Royal Prince Alfred Hospital, Sydney, NSW, Australia.

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http://dx.doi.org/10.1111/resp.13909DOI Listing

The Associations of Interstitial Lung Abnormalities with Cancer Diagnoses and Mortality.

Eur Respir J 2020 Jul 9. Epub 2020 Jul 9.

University of Iceland, Faculty of Medicine, Reykjavik, Iceland.

An increased incidence of lung cancer is well-known among patients with idiopathic pulmonary fibrosis. It is unknown whether interstitial lung abnormalities, early fibrotic changes of the lung, are a risk factor for lung cancer in the general population.The study's objective was to assess whether interstitial lung abnormalities were associated with diagnoses of, and mortality from, lung cancer and other cancers. Read More

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http://dx.doi.org/10.1183/13993003.02154-2019DOI Listing

Metabolic activation of pirfenidone mediated by cytochrome P450s and sulfotransferases.

J Med Chem 2020 Jul 9. Epub 2020 Jul 9.

Pirfenidone is approved for the treatment of idiopathic pulmonary fibrosis. Idiosyncratic drug reactions, due to clinical application of pirfenidone, have been documented, even along with death cases resulting from acute liver failure. The present study aimed at the investigation of metabolic activation of pirfenidone possibly participating in the reported adverse reactions. Read More

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http://dx.doi.org/10.1021/acs.jmedchem.9b02073DOI Listing
July 2020
5.447 Impact Factor

Management of interstitial lung diseases: A consensus statement of the Indian Chest Society (ICS) and National College of Chest Physicians (NCCP).

Lung India 2020 Jul-Aug;37(4):359-378

Department of Medicine, University of Washington, Seattle, USA.

Background: Interstitial lung disease (ILD) is a complex and heterogeneous group of acute and chronic lung diseases of several known and unknown causes. While clinical practice guidelines (CPG) for idiopathic pulmonary fibrosis (IPF) have been recently updated, CPG for ILD other than IPF are needed.

Methods: A working group of multidisciplinary clinicians familiar with clinical management of ILD (pulmonologists, radiologist, pathologist, and rheumatologist) and three epidemiologists selected by the leaderships of Indian Chest Society and National College of Chest Physicians, India, posed questions to address the clinically relevant situation. Read More

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http://dx.doi.org/10.4103/lungindia.lungindia_275_20DOI Listing

A Fatal Case of Coronavirus Disease 2019 (COVID-19) in a Patient With Idiopathic Pulmonary Fibrosis.

Cureus 2020 Jun 3;12(6):e8432. Epub 2020 Jun 3.

Cardiology, Decatur Memorial Hospital, Decatur, USA.

The number of cases of coronavirus disease 2019 (COVID-19) has been exponentially increasing everyday. It is important to recognize the comorbidities and risk factors associated with this highly contagious and serious disease that has caused thousands of deaths worldwide. Patients with certain conditions like diabetes, hypertension, cardiovascular disease and chronic lung diseases have been reported to develop serious complications from COVID-19. Read More

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http://dx.doi.org/10.7759/cureus.8432DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7336650PMC

Comprehensive analysis of lncRNA-associated competing endogenous RNA network and immune infiltration in idiopathic pulmonary fibrosis.

J Thorac Dis 2020 May;12(5):1856-1865

Department of Infectious Diseases, Shandong University Qilu Hospital, Jinan 250012, China.

Background: Idiopathic pulmonary fibrosis (IPF) is a life-threatening lung disorder with an unknown aetiology. The roles of long non-coding RNAs (lncRNAs) and its related competing endogenous RNAs (ceRNA) network in IPF remains poorly understood. In this study, we aimed to build a lncRNA-miRNA-mRNA network and explore the pathogenesis of IPF. Read More

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http://dx.doi.org/10.21037/jtd-19-2842DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7330328PMC

The ageing lung under stress.

Eur Respir Rev 2020 Jun 7;29(156). Epub 2020 Jul 7.

Comprehensive Pneumology Center (CPC), University Hospital of the Ludwig-Maximilians-University (LMU) and Helmholtz Zentrum München, Munich, Germany

Healthy ageing of the lung involves structural changes but also numerous cell-intrinsic and cell-extrinsic alterations. Among them are the age-related decline in central cellular quality control mechanisms such as redox and protein homeostasis. In this review, we would like to provide a conceptual framework of how impaired stress responses in the ageing lung, as exemplified by dysfunctional redox and protein homeostasis, may contribute to onset and progression of COPD and idiopathic pulmonary fibrosis (IPF). Read More

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http://dx.doi.org/10.1183/16000617.0126-2020DOI Listing

Paracrine SPARC signaling dysregulates alveolar epithelial barrier integrity and function in lung fibrosis.

Cell Death Discov 2020 30;6:54. Epub 2020 Jun 30.

Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, SO16 6YD UK.

Idiopathic pulmonary fibrosis (IPF) is a chronic scarring disease in which aging, environmental exposure(s) and genetic susceptibility have been implicated in disease pathogenesis, however, the causes and mechanisms of the progressive fibrotic cascade are still poorly understood. As epithelial-mesenchymal interactions are essential for normal wound healing, through human 2D and 3D in vitro studies, we tested the hypothesis that IPF fibroblasts (IPFFs) dysregulate alveolar epithelial homeostasis. Conditioned media from IPFFs exaggerated the wound-healing response of primary human Type II alveolar epithelial cells (AECs). Read More

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http://dx.doi.org/10.1038/s41420-020-0289-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7327077PMC

Integrin alpha-5 silencing leads to myofibroblastic differentiation in IPF-derived human lung fibroblasts.

Ther Adv Chronic Dis 2020 24;11:2040622320936023. Epub 2020 Jun 24.

Pulmonary Department, Meir Medical Center, Kfar Saba, Israel Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.

Background And Objective: The term 'fibroblast' covers a heterogeneous cell population in idiopathic pulmonary fibrosis (IPF). The fibroblasts are considered as main effector cells, because they promote disease progression by releasing exaggerated amounts of extracellular matrix proteins and modifying cell microenvironment. As IPF-derived human lung fibroblasts (IPF-HLFs) were shown to express higher levels of integrin alpha-5 (ITGA5) than normal derived HLFs (N-HLFs), we explored the importance of ITGA5 to IPF progression. Read More

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http://dx.doi.org/10.1177/2040622320936023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7315658PMC

Single-cell RNA sequencing reveals that lung mesenchymal progenitor cells in IPF exhibit pathological features early in their differentiation trajectory.

Sci Rep 2020 Jul 7;10(1):11162. Epub 2020 Jul 7.

University of Minnesota, Department of Medicine, Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, Minneapolis, USA.

In Idiopathic Pulmonary Fibrosis (IPF), there is unrelenting scarring of the lung mediated by pathological mesenchymal progenitor cells (MPCs) that manifest autonomous fibrogenicity in xenograft models. To determine where along their differentiation trajectory IPF MPCs acquire fibrogenic properties, we analyzed the transcriptome of 335 MPCs isolated from the lungs of 3 control and 3 IPF patients at the single-cell level. Using transcriptional entropy as a metric for differentiated state, we found that the least differentiated IPF MPCs displayed the largest differences in their transcriptional profile compared to control MPCs. Read More

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http://dx.doi.org/10.1038/s41598-020-66630-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7341888PMC

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features.

Diagnostics (Basel) 2020 Jul 3;10(7). Epub 2020 Jul 3.

Regional Centre for Interstitial and Rare Lung Disease, Department of Clinical and Molecular Biomedicine, University of Catania, 95123 Catania, Italy.

Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Read More

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http://dx.doi.org/10.3390/diagnostics10070450DOI Listing

Development and Initial Validation Analyses of the Living with Idiopathic Pulmonary Fibrosis (L-IPF) Questionnaire.

Am J Respir Crit Care Med 2020 Jul 7. Epub 2020 Jul 7.

Endpoint Outcomes, 455680, Boston, Massachusetts, United States.

Rationale: Several new drugs for idiopathic pulmonary fibrosis (IPF) are in development. Tools are needed to assess whether these drugs benefit patients on outcomes that matter most to them. Health-related quality of life (HRQL) is one such outcome. Read More

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http://dx.doi.org/10.1164/rccm.202002-0415OCDOI Listing

CMH-Small Molecule Docks into SIRT1, Elicits Human IPF-Lung Fibroblast Cell Death, Inhibits Ku70-deacetylation, FLIP and Experimental Pulmonary Fibrosis.

Biomolecules 2020 Jul 2;10(7). Epub 2020 Jul 2.

Lung Cellular and Molecular Biology Laboratory, Institute of Pulmonary Medicine, Hadassah-Hebrew University Medical Center, POB 12000, Jerusalem 91120, Israel.

Regenerative capacity in vital organs is limited by fibrosis propensity. Idiopathic pulmonary fibrosis (IPF), a progressive lung disease linked with aging, is a classic example. In this study, we show that in flow cytometry, immunoblots (IB) and in lung sections, FLIP levels can be regulated, in vivo and in vitro, through SIRT1 activity inhibition by CMH (4-(4-Chloro-2-methylphenoxy)-N-hydroxybutanamide), a small molecule that, as we determined here by structural biology calculations, docked into its nonhistone substrate Ku70-binding site. Read More

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http://dx.doi.org/10.3390/biom10070997DOI Listing

Nintedanib Reduces Neutrophil Chemotaxis via Activating GRK2 in Bleomycin-Induced Pulmonary Fibrosis.

Int J Mol Sci 2020 Jul 2;21(13). Epub 2020 Jul 2.

Department of Chest Medicine, Taipei Veterans General Hospital, Taipei 112, Taiwan.

Neutrophils are involved in the alveolitis of idiopathic pulmonary fibrosis (IPF). However, their pathogenic mechanisms are still poorly understood. Nintedanib has antifibrotic and anti-inflammatory activity in IPF. Read More

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http://dx.doi.org/10.3390/ijms21134735DOI Listing

Genetic Variation in CCL18 Gene Influences CCL18 Expression and Correlates with Survival in Idiopathic Pulmonary Fibrosis-Part B.

J Clin Med 2020 Jun 25;9(6). Epub 2020 Jun 25.

Department of Respiratory Medicine, Hannover Medical School and Biomedical Research in End-stage and Obstructive Lung Disease Hannover, German Lung Research Center (DZL), 30265 Hannover, Germany.

Idiopathic pulmonary fibrosis (IPF) is a progressive disease with high mortality. CC-chemokine ligand 18 (CCL18) is predictive of survival in IPF. We described correlation of CCL18 serum levels with the genotype of C > T polymorphism the -gene, which was associated with survival in a pre-antifibrotic cohort (Part-A). Read More

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http://dx.doi.org/10.3390/jcm9061993DOI Listing

Transcriptomic changes involved in the dedifferentiation of myofibroblasts derived from the lung of a patient with idiopathic pulmonary fibrosis.

Mol Med Rep 2020 Aug 10;22(2):1518-1526. Epub 2020 Jun 10.

Department of Biomedical Science, Graduate School of Medicine, Chiba University, Chuo‑ku, Chiba 260‑8670, Japan.

Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease of unknown etiology. Under pathological conditions in lungs with IPF, myofibroblasts serve a key role in fibrogenesis via the accumulation of an excessive amount of extracellular matrix. To develop effective therapeutic interventions against IPF, studies have recently focused on how to dedifferentiate established myofibroblasts. Read More

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http://dx.doi.org/10.3892/mmr.2020.11218DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7339813PMC

Time to diagnosis of idiopathic pulmonary fibrosis in the IPF-PRO Registry.

BMJ Open Respir Res 2020 Jul;7(1)

Medical University of South Carolina, Charleston, South Carolina, USA.

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with high mortality. Patient characteristics associated with diagnostic delays are not well described.

Methods: Subjects who had not been diagnosed with IPF prior to referral and received a new diagnosis of IPF at an enrolling centre for the IPF-PRO (diopathic ulmonary ibrosis spective Outcomes) Registry were characterised as having a longer (>1 year) or shorter (≤1 year) time from symptom onset to diagnosis and from first imaging evidence of fibrosis to diagnosis. Read More

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http://dx.doi.org/10.1136/bmjresp-2020-000567DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7337884PMC

Phosphodiesterase isoforms and cAMP compartments in the development of new therapies for obstructive pulmonary diseases.

Curr Opin Pharmacol 2020 Jul 1;51:34-42. Epub 2020 Jul 1.

Department of Biomedical and Pharmaceutical Sciences, Chapman University School of Pharmacy, Irvine, CA, USA. Electronic address:

The second messenger molecule 3'5'-cyclic adenosine monophosphate (cAMP) imparts several beneficial effects in lung diseases such as asthma, chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). While cAMP is bronchodilatory in asthma and COPD, it also displays anti-fibrotic properties that limit fibrosis. Phosphodiesterases (PDEs) metabolize cAMP and thus regulate cAMP signaling. Read More

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http://dx.doi.org/10.1016/j.coph.2020.05.002DOI Listing

Association of serum macrophage-mannose receptor CD206 with mortality in idiopathic pulmonary fibrosis.

Int Immunopharmacol 2020 Jul 1;86:106732. Epub 2020 Jul 1.

Department of Respiratory Medicine, Nanjing Drum Tower Hospital Clinical College of Nanjing Medical University, No. 321 Zhongshan Road, Nanjing 210008, Jiangsu, People's Republic of China; Department of Respiratory Medicine, The Affiliated Drum Tower Hospital of Nanjing University Medical School, No. 321 Zhongshan Road, Nanjing 210008, Jiangsu, People's Republic of China. Electronic address:

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is attracting considerable attention due to disease acceleration and substantial mortality. Macrophages are known to regulate the fibrotic process in idiopathic pulmonary fibrosis.

Objective: We investigated if two new macrophage-specific serum biomarkers, soluble mannose receptor (MR, sCD206) and soluble CD163 (sCD163), increased in serum obtained from patients with AE-IPF compared to stable IPF (S-IPF). Read More

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http://dx.doi.org/10.1016/j.intimp.2020.106732DOI Listing

Autophagy in chronic lung disease.

Prog Mol Biol Transl Sci 2020 12;172:135-156. Epub 2020 Mar 12.

NewYork-Presbyterian Hospital, Weill Cornell Medicine, New York, NY, United States; Division of Nephrology and Hypertension, Joan and Sanford I. Weill Department of Medicine, Weill Cornell Medicine, New York, NY, United States. Electronic address:

The development of chronic lung disease occurs as a consequence of multiple cellular events that involve an initial insult which often leads to the development of chronic inflammation, and the dysregulation of cellular proliferation and cell death mechanisms. Multiple cell types in the lung are key to the respiratory and protective/barrier functions necessary to manage the chronic exposures to environmental, mechanical, and oxidative stressors. Autophagy is essential to lung development and homeostasis, as well as the prevention and development of disease. Read More

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http://dx.doi.org/10.1016/bs.pmbts.2020.02.001DOI Listing

Effects of pirfenidone targeting the tumor microenvironment and tumor-stroma interaction as a novel treatment for non-small cell lung cancer.

Sci Rep 2020 Jul 2;10(1):10900. Epub 2020 Jul 2.

Department of General Thoracic Surgery, Osaka University Graduate School of Medicine, 2-2-L5 Yamadaoka, Suita, Osaka, 565-0871, Japan.

Targeting cancer-associated fibroblasts (CAFs), as well as the crosstalk between stroma and cancer cells, could be of value in managing cancers. Pirfenidone (PFD) is an anti-fibrotic agent for idiopathic pulmonary fibrosis. This study aimed to investigate the possibility that PFD might exert an anti-tumor effect through inhibition of fibroblast activation and the tumor-stroma interaction in non-small cell lung cancer (NSCLC) cell lines in vitro and in vivo. Read More

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http://dx.doi.org/10.1038/s41598-020-67904-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7331721PMC

[Relationship between Cellular Microenvironment and Idiopathic Pulmonary Fibrosis].

Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2020 Jun;42(3):410-416

First Clinical Medical College,Zhejiang University of Traditional Chinese Medicine,Hangzhou 310053,China.

Idiopathic pulmonary fibrosis(IPF)is a progressive lung disease characterized by pulmonary interstitial fibrosis and pulmonary dysfunction.Cell microenvironment is mainly composed of cell components,extracellular matrix,extracellular regulators,and liquid substances.Changes in microenvironment components are closely related to IPF. Read More

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http://dx.doi.org/10.3881/j.issn.1000-503X.11249DOI Listing

Histopathologic Assessment of Suspected Idiopathic Pulmonary Fibrosis: Where We Are and Where We Need to Go.

Arch Pathol Lab Med 2020 Jul 2. Epub 2020 Jul 2.

and the Department of Pathology, Mount Sinai Health System, Icahn School of Medicine, New York, New York (Dr Beasley).

Context.—: Accurate diagnosis of idiopathic pulmonary fibrosis (IPF) requires multidisciplinary diagnosis that includes clinical, radiologic, and often pathologic assessment. In 2018, the American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and the Latin American Thoracic Society (ATS/ERS/JRS/ALAT) and the Fleischner Society each published guidelines for the diagnosis of IPF, which include criteria for 4 categories of confidence of a histologic usual interstitial pneumonia (UIP) pattern. Read More

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http://dx.doi.org/10.5858/arpa.2020-0052-RADOI Listing

Patients with idiopathic pulmonary fibrosis: Overcoming 'geographic isolation'.

Respirology 2020 Jul 2. Epub 2020 Jul 2.

Respiratory Medicine, Royal Brompton Hospital, London, UK.

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http://dx.doi.org/10.1111/resp.13883DOI Listing

Krebs von den Lungen-6 as biomarker for disease severity assessment in interstitial lung disease: a comprehensive review.

Biomark Med 2020 Jul 2. Epub 2020 Jul 2.

Respiratory Diseases & Lung Transplantation, Department of Medical & Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy.

Interstitial lung diseases (ILD) are a group of lung disorders characterized by interstitial lung thickening. Krebs von den Lungen-6 (KL-6) is a molecule that is predominantly expressed by damaged alveolar type II cells and it has been proposed as a potential biomarker of different ILD. A growing literature about KL-6 has been reviewed and selected to evaluate its role in the clinical management of ILD to predict disease diagnosis, activity, prognosis and treatment response. Read More

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http://dx.doi.org/10.2217/bmm-2019-0545DOI Listing

Incidence and impact of extra-pulmonary organ failures on hospital mortality in acute exacerbation of idiopathic pulmonary fibrosis.

Sci Rep 2020 Jul 1;10(1):10742. Epub 2020 Jul 1.

Department of Pulmonary and Critical Care Medicine, Nanjing Drum Tower Hospital, Clinical College of Nanjing Medical School, No. 321 Zhongshan Road, Nanjing, 210008, Jiangsu, China.

To evaluate hospital mortality and associated risk factors for acute exacerbations of idiopathic pulmonary fibrosis (AEIPF). Emphases were put on incidence and impact of extra-pulmonary organ failures. Patients diagnosed with AEIPF from July 2014 to September 2018 were enrolled. Read More

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http://dx.doi.org/10.1038/s41598-020-67598-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7329823PMC

Utility of illness severity scores to predict mortality in patients hospitalised with respiratory deterioration of Idiopathic Pulmonary Fibrosis.

QJM 2020 Jul 1. Epub 2020 Jul 1.

Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Southmead Hospital, Bristol, UK, BS10 5NB.

Introduction: In the context of IPF, respiratory related admissions to hospital are associated with a high morbidity and short-term mortality with significant burden on secondary care services. It has yet to be determined how to accurately identify patients at risk of acute respiratory deterioration (ARD) or the prognosticating factors.

Objective: We sought to define the characteristics of hospitalised ARD-IPF patients in a real-world cohort and investigate factors associated with worse outcomes. Read More

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http://dx.doi.org/10.1093/qjmed/hcaa214DOI Listing

Transcriptomic profiling reveals disease-specific characteristics of epithelial cells in idiopathic pulmonary fibrosis.

Respir Res 2020 Jun 30;21(1):165. Epub 2020 Jun 30.

Department of Biomedicine, University Hospital Basel, University of Basel, Hebelstrasse 20, CH-4031, Basel, Switzerland.

Background: Idiopathic pulmonary fibrosis (IPF) is an incurable disease characterized by progressive lung fibrosis ultimately resulting in respiratory failure and death. Recurrent micro-injuries to the alveolar epithelium and aberrant alveolar wound healing with impaired re-epithelialization define the initial steps of the pathogenic trajectory. Failure of timely alveolar epithelial repair triggers hyper-proliferation of mesenchymal cells accompanied by increased deposition of extracellular matrix into the lung interstitium. Read More

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http://dx.doi.org/10.1186/s12931-020-01414-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7329456PMC

Discovery of Novel Selective and Orally Bioavailable Phosphodiesterase-1 Inhibitors for the Efficient Treatment of Idiopathic Pulmonary Fibrosis.

J Med Chem 2020 Jun 30. Epub 2020 Jun 30.

School of Pharmaceutical Sciences, Sun Yat-sen University, Guangzhou 510006, P. R. China.

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and devastating lung disease lacking effective therapy. To identify whether phosphodiesterase-1 (PDE1) inhibition could act as a novel target for the treatment of IPF, hit-to-lead structural optimizations were performed on the PDE9/PDE1 dual inhibitor ()-, leading to compound with an IC of 2.9 nM against PDE1C, excellent selectivity across PDE subfamilies, reasonable drug-like properties, and remarkable pharmacodynamic effects as an anti-IPF agent. Read More

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http://dx.doi.org/10.1021/acs.jmedchem.0c00711DOI Listing
June 2020
5.447 Impact Factor

Chronic Hypersensitivity Pneumonitis, an Interstitial Lung Disease with Distinct Molecular Signatures.

Am J Respir Crit Care Med 2020 Jun 30. Epub 2020 Jun 30.

University of Colorado, School of Medicine, Department of Medicine, Aurora, Colorado, United States;

Rationale: Chronic hypersensitivity pneumonitis (CHP) is caused by an immune response to antigen inhalation and is characterized by variable histopathological and clinical features. A subset of CHP patients have usual interstitial pneumonia and appear to be clinically similar to patients with idiopathic pulmonary fibrosis (IPF).

Objectives: To determine the common and unique molecular features of CHP and IPF. Read More

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http://dx.doi.org/10.1164/rccm.202001-0134OCDOI Listing

Identification and functional characterization of a novel surfactant protein A2 mutation (p.N207Y) in a Chinese family with idiopathic pulmonary fibrosis.

Mol Genet Genomic Med 2020 Jun 30:e1393. Epub 2020 Jun 30.

Department of Respiratory Medicine, Diagnosis and Treatment Center of Respiratory Disease, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China.

Background: Idiopathic pulmonary fibrosis (IPF) is a serious disorder with a high mortality rate worldwide. It is characterized by irreversible scarring of the lung parenchyma resulting from excessive collagen production by proliferating fibroblasts/myofibroblasts. Previous studies have revealed that mutations in surfactant protein-related genes and telomerase complex genes are crucial underlying genetic factors. Read More

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http://dx.doi.org/10.1002/mgg3.1393DOI Listing

Acute exacerbation of unclassifiable idiopathic interstitial pneumonia: comparison with idiopathic pulmonary fibrosis.

Ther Adv Respir Dis 2020 Jan-Dec;14:1753466620935774

Second Division, Department of Internal Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan.

Background: Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is well known as a life-threatening condition during its clinical course. However, the clinical features and prognosis in AE of unclassifiable idiopathic interstitial pneumonia (AE-UCIIP) remain to be elucidated. The aim of this study was to clarify the clinical features and prognosis of AE-UCIIP compared with those of AE-IPF. Read More

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http://dx.doi.org/10.1177/1753466620935774DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7328360PMC

[Differential diagnosis of idiopathic pulmonary fibrosis].

Ter Arkh 2020 Apr 27;92(3):102-108. Epub 2020 Apr 27.

Central Research Institute of Tuberculosis.

The review is devoted to the urgent problem of modern pulmonology: the differential diagnosis of idiopathic pulmonary fibrosis (ILF). ILF occupies a special place among many interstitial lung diseases for a number of reasons: 1) it is a deadly disease; 2) early diagnosis and adequate antifibrotic therapy significantly extend the life expectancy of patients; 3) anti-inflammatory drugs (corticosteroids) and cytostatics with ILF that are widely used in other forms of interstitial lung diseases are ineffective and accelerate the progression of the process; 4) the commonality of the main clinical signs (increasing respiratory failure) of various interstitial lung diseases. The list of respiratory diseases with which ILF should be differentiated is huge, and if with diffuse lung lesions of a known nature (disseminated pulmonary tuberculosis, pneumoconiosis, etc. Read More

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http://dx.doi.org/10.26442/00403660.2020.03.000346DOI Listing

[Exacerbation of idiopathic pulmonary fibrosis].

Ter Arkh 2020 Apr 27;92(3):73-77. Epub 2020 Apr 27.

Sechenov First Moscow State Medical University (Sechenov University).

Idiopathic pulmonary fibrosis (IPF) is usually characterized by a chronic and slowly progressive course. According to several studies, a small number of patients with IPF (about 515%) develops an acute deterioration of deasese exacerbation of IPF. Exacerbations of IPF can occur at any time of the disease and sometimes becomes the first manifestation of IPF. Read More

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http://dx.doi.org/10.26442/00403660.2020.03.000402DOI Listing

Impact of idiopathic pulmonary fibrosis on recurrence after surgical treatment for stage I-III non-small cell lung cancer.

PLoS One 2020 29;15(6):e0235126. Epub 2020 Jun 29.

Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, South Korea.

Background: Idiopathic pulmonary fibrosis (IPF) is an independent risk factor for lung cancer (LC) development; however, its effect on recurrence after curative surgery remains unclear.

Objectives: This study aimed to determine the impact of IPF on recurrence-free survival following curative surgical resection of stage I-III non-small cell lung cancer (NSCLC) and investigate the effects of patient and surgical factors on the risk of recurrence.

Methods: We reviewed retrospectively collected data of patients with surgically resected stage I-III NSCLC from two tertiary care hospitals in South Korea. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0235126PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7323957PMC

Nanodomains in cardiopulmonary disorders and the impact of air pollution.

Biochem Soc Trans 2020 Jun 29. Epub 2020 Jun 29.

Department of Molecular Pharmacology, University of Groningen, Groningen, The Netherlands.

Air pollution is a major environmental threat and each year about 7 million people reported to die as a result of air pollution. Consequently, exposure to air pollution is linked to increased morbidity and mortality world-wide. Diesel automotive engines are a major source of urban air pollution in the western societies encompassing particulate matter and diesel exhaust particles (DEP). Read More

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http://dx.doi.org/10.1042/BST20190250DOI Listing

Reprogramming of profibrotic macrophages for treatment of bleomycin-induced pulmonary fibrosis.

EMBO Mol Med 2020 Jun 29:e12034. Epub 2020 Jun 29.

Department of Chemistry and Institute for Drug Discovery, Purdue University, West Lafayette, IN, USA.

Fibrotic diseases cause organ failure that lead to ~45% of all deaths in the United States. Activated macrophages stimulate fibrosis by secreting cytokines that induce fibroblasts to synthesize collagen and extracellular matrix proteins. Although suppression of macrophage-derived cytokine production can halt progression of fibrosis, therapeutic agents that prevent release of these cytokines (e. Read More

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http://dx.doi.org/10.15252/emmm.202012034DOI Listing

Toll interacting protein protects bronchial epithelial cells from bleomycin-induced apoptosis.

FASEB J 2020 Jun 28. Epub 2020 Jun 28.

Division of Pulmonary, Allergy and Critical Care Medicine and the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease, University of Pittsburgh, Pittsburgh, PA, USA.

Idiopathic pulmonary fibrosis (IPF) is characterized by altered epithelial cell phenotypes, which are associated with myofibroblast accumulation in the lung. Atypical alveolar epithelial cells in IPF express molecular markers of airway epithelium. Polymorphisms within and around Toll interacting protein (TOLLIP) are associated with the susceptibility to IPF and mortality. Read More

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http://dx.doi.org/10.1096/fj.201902636RRDOI Listing

Integrated Analysis of a Gene Correlation Network Identifies Critical Regulation of Fibrosis by lncRNAs and TFs in Idiopathic Pulmonary Fibrosis.

Biomed Res Int 2020 2;2020:6537462. Epub 2020 Jun 2.

Traditional Chinese Medicine Hospital Affiliated to Xinjiang Medical University & National Clinical Research Base of Traditional Chinese Medicine, Urumqi, Xinjiang 830000, China.

Idiopathic pulmonary fibrosis (IPF), the most frequent form of irreversible interstitial pneumonia with unknown etiology, is characterized by massive remodeling of lung architecture and followed by progressive loss of lung function. However, the key regulatory genes and the specific signaling pathways involved in the onset and progression of IPF still remain unclear. The present study is aimed at investigating the key role of long noncoding RNAs (lncRNAs) and transcription factors (TFs) involved in the pathogenesis of IPF through the integrated analysis of three gene expression profiles from the GEO dataset (GSE2052, GSE44723, and GSE24206). Read More

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http://dx.doi.org/10.1155/2020/6537462DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7290873PMC

Human Fibrosis: Is There Evidence for a Genetic Predisposition in Musculoskeletal Tissues?

J Arthroplasty 2020 Jun 4. Epub 2020 Jun 4.

Department of Orthopedic Surgery, Mayo Clinic, Rochester, MN.

Background: Pathologic fibrosis is characterized by dysregulation of gene expression with excessive extracellular matrix production. The genetic basis for solid organ fibrosis is well described in the literature. However, there is a paucity of evidence for similar processes in the musculoskeletal (MSK) system. Read More

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http://dx.doi.org/10.1016/j.arth.2020.05.070DOI Listing