8,758 results match your criteria Pulmonary Fibrosis Idiopathic


Physiological Responses and Prognostic Value of Common Exercise Testing Modalities in Idiopathic Pulmonary Fibrosis.

J Cardiopulm Rehabil Prev 2019 May;39(3):193-198

Master of Cancer Care Program, School of Health Sciences, Saint Francis University, Loretto, Pennsylvania (Dr Vainshelboim); Pulmonary Institute, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Israel (Drs Vainshelboim, Izhakian, Unterman, and Kramer); Research Center in Physical Activity, Health and Leisure (CIAFEL), Faculty of Sport, University of Porto, Portugal (Drs Vainshelboim and Oliveira); Cardiology Division, Veterans Affairs Palo Alto Health Care System/Stanford University, California (Dr Myers); Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel (Drs Izhakian, Unterman, and Kramer).

Purpose: This pilot study aimed to compare physiological responses between cycle cardiopulmonary exercise tests (CPETs) and 6-min walk tests (6MWTs) and to assess their prognostic value among patients with idiopathic pulmonary fibrosis (IPF).

Methods: Thirty-four patients with IPF (68 ± 8 yr) underwent CPETs and 6MWTs and were followed up for 40 mo. Differences, levels of agreement, and relative risks for mortality were analyzed between measured and estimated peak responses for the 2 tests. Read More

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http://dx.doi.org/10.1097/HCR.0000000000000362DOI Listing

Apoptosis resistance in fibroblasts precedes progressive scarring in pulmonary fibrosis and is partially mediated by Toll-like receptor 4 activation.

Toxicol Sci 2019 Apr 24. Epub 2019 Apr 24.

Department of Environmental Medicine, University of Rochester Medical Center, NY, USA.

Inhalation of environmental toxicants such as cigarette smoke, metal or wood dust, silica, or asbestos is associated with increased risk for idiopathic pulmonary fibrosis (IPF). IPF involves progressive scarring of lung tissue, which interferes with normal respiration and is ultimately fatal; however, the complex cellular mechanisms of IPF pathogenesis remain unclear. Fibroblast apoptosis is essential in normal wound healing but is dysregulated in IPF. Read More

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http://dx.doi.org/10.1093/toxsci/kfz103DOI Listing

Cellular senescence and senescence-associated secretory phenotype: comparison of idiopathic pulmonary fibrosis, connective tissue disease-associated interstitial lung disease, and chronic obstructive pulmonary disease.

J Thorac Dis 2019 Mar;11(3):857-864

Department of Pathology, Yokohama City University Graduate School of Medicine, Yokohama, Japan.

Background: The senescence-associated secretory phenotype (SASP) develops due to cellular senescence during conditions such as chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). However, studies comparing the degree of cellular senescence and SASP between COPD and IPF are limited. Furthermore, to the best of our knowledge, no study has examined cellular senescence and/or SASP in connective tissue disease-associated interstitial lung disease (CTD-ILD). Read More

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http://dx.doi.org/10.21037/jtd.2019.02.11DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6462683PMC

Comment on 'Fatal, incidental, idiopathic pulmonary fibrosis in a patient receiving long-term low-dose methotrexate for psoriasis': reply from author.

Clin Exp Dermatol 2019 Apr 24. Epub 2019 Apr 24.

Respiratory Medicine, Ninewells Hospital, Dundee, UK.

There appears to be some overlap in pathogenetic mechanisms between psoriasis and idiopathic pulmonary fibrosis e.g. raised IL-17; indeed, the possibility of a restrictive form of psoriatic lung disease has been raised although it appears to be rare. Read More

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http://dx.doi.org/10.1111/ced.13993DOI Listing

Clinical Pharmacokinetics and Pharmacodynamics of Nintedanib.

Clin Pharmacokinet 2019 Apr 23. Epub 2019 Apr 23.

Translational Medicine and Clinical Pharmacology, Boehringer Ingelheim Pharma GmbH & Co. KG, Birkendorfer Strasse 65, 88397, Biberach an der Riss, Germany.

Nintedanib is an oral, small-molecule tyrosine kinase inhibitor approved for the treatment of idiopathic pulmonary fibrosis and patients with advanced non-small cell cancer of adenocarcinoma tumour histology. Nintedanib competitively binds to the kinase domains of vascular endothelial growth factor (VEGF), platelet-derived growth factor (PDGF) and fibroblast growth factor (FGF). Studies in healthy volunteers and in patients with advanced cancer have shown that nintedanib has time-independent pharmacokinetic characteristics. Read More

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http://dx.doi.org/10.1007/s40262-019-00766-0DOI Listing

Pulmonary Rehabilitation for Exercise Tolerance and Quality of Life in IPF Patients: A Systematic Review and Meta-Analysis.

Biomed Res Int 2019 21;2019:8498603. Epub 2019 Mar 21.

Collaborative Innovation Center for Respiratory Disease Diagnosis and Treatment & Chinese Medicine Development of Henan Province, Henan University of Chinese Medicine, Zhengzhou, Henan 450046, China.

Objective: The aim of this study is to evaluate the efficacy and safety of pulmonary rehabilitation (PR) in patients with idiopathic pulmonary fibrosis (IPF). s. Embase, PubMed, Cochrane Library, China National Knowledge Infrastructure (CNKI), Chongqing VIP (CQVIP), Wanfang Data, and Chinese Biomedical Literature Database (SinoMed) were comprehensively searched. Read More

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http://dx.doi.org/10.1155/2019/8498603DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6448340PMC
March 2019
3 Reads

Recommended Reading from the Massachusetts General Hospital/ Beth Israel Deaconess Medical Center/ Harvard Pulmonary and Critical Care Medicine Fellows.

Am J Respir Crit Care Med 2019 Apr 23. Epub 2019 Apr 23.

Beth Israel Deaconess Medical Center, 1859, Pulmonary and Critical Care Medicine, Boston, Massachusetts, United States ;

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http://dx.doi.org/10.1164/rccm.201902-0471RRDOI Listing

The Effects of Epigallocatechin Gallate (EGCG) on Pulmonary Fibroblasts of Idiopathic Pulmonary Fibrosis (IPF)-A Next-Generation Sequencing and Bioinformatic Approach.

Int J Mol Sci 2019 Apr 22;20(8). Epub 2019 Apr 22.

Graduate Institute of Clinical Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan.

Idiopathic pulmonary fibrosis (IPF) is a disabling and lethal chronic progressive pulmonary disease. Epigallocatechin gallate (EGCG) is a polyphenol, which is the major biological component of green tea. The anti-oxidative, anti-inflammatory, and anti-fibrotic effects of EGCG have been shown in some studies, whereas its effects in altering gene expression in pulmonary fibroblasts have not been systematically investigated. Read More

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http://dx.doi.org/10.3390/ijms20081958DOI Listing

Clinical features and prognosis of microscopic polyangiitis with usual interstitial pneumonia compared with idiopathic pulmonary fibrosis.

Clin Respir J 2019 Apr 23. Epub 2019 Apr 23.

Department of Respiratory and Critical Care Medicine, Beijing, Key Laboratory of Respiratory and Pulmonary Circulation Disorders, Beijing, Chao-Yang Hospital-Beijing Institute of Respiratory Medicine, Capital Medical University, No. 8 Gongren Tiyuchang South Road, Beijing, 100020, P.R. China.

Introduction: Pulmonary involvement in microscopic polyangiitis (MPA) is common, little is known about the clinical features of MPA with interstitial pneumonia (MPA-IP).

Objectives: This study aimed to explore the prevalence of microscopic polyangiitis associated usual interstitial pneumonia (UIP)(MPA-UIP) and compare its clinical features and prognosis with those of MPA-non-UIP and idiopathic pulmonary fibrosis (IPF).

Methods: A total of 73 patients with MPA-IP were identified and divided into MPA-UIP patients and MPA-non-UIP patients. Read More

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http://dx.doi.org/10.1111/crj.13032DOI Listing

Safety, Pharmacokinetics, and Pharmacodynamics of the Autotaxin Inhibitor GLPG1690 in Healthy Subjects: Phase 1 Randomized Trials.

J Clin Pharmacol 2019 Apr 23. Epub 2019 Apr 23.

Galapagos Biotech Limited, Cambridge, United Kingdom.

GLPG1690 is a novel autotaxin inhibitor in development for the treatment of idiopathic pulmonary fibrosis (IPF). We report phase 1 studies investigating the safety, tolerability, pharmacokinetics (PK), and pharmacodynamics (PD) of GLPG1690 in healthy subjects. We performed a first-in-human randomized, double-blind, placebo-controlled trial of single (20, 60, 150, 300, 600, 1000, 1500 mg) and multiple (14 days: 150 mg twice daily; 600 and 1000 mg once daily) ascending oral doses of GLPG1690 (NCT02179502), and a randomized, open-label, crossover relative bioavailability study to compare the PK of tablet and capsule formulations of GLPG1690 600 mg and to assess the effect of food on PK of the tablet formulation (NCT03143712). Read More

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http://dx.doi.org/10.1002/jcph.1424DOI Listing

Elevated levels of periostin and TGF-β1 in the bronchoalveolar lavage fluid of patients with idiopathic eosinophilic pneumonia.

Asian Pac J Allergy Immunol 2019 Apr 23. Epub 2019 Apr 23.

Department of Respiratory Medicine, Kawasaki Medical School, Okayama.

Background: Periostin is induced in bronchial epithelial cells and fibroblasts by various stimuli including interleukin (IL)13 and transforming growth factor (TGF)-β1, and is involved in allergic diseases such as asthma and atopic dermatitis, playing an important role in tissue remodeling and fibrosis. The role of periostin in the pathogenesis of eosinophilic lung diseases, however, is unclear.

Objective: To examine the contribution of periostin to eosinophilic inflammation of the lung in humans, we evaluated periostin, IL-13, and TGF-β1 levels in the bronchoalveolar lavage fluid (BALF) of patients with eosinophilic pneumonia (EP). Read More

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http://dx.doi.org/10.12932/AP-111018-0414DOI Listing

The Role of Pathological Aging in Cardiac and Pulmonary Fibrosis.

Aging Dis 2019 Apr 1;10(2):419-428. Epub 2019 Apr 1.

1School of Medicine and Public Health, The University of Newcastle, Callaghan, NSW, Australia.

Aging promotes a range of degenerative pathologies characterized by progressive losses of tissue and/or cellular function. Fibrosis is the hardening, overgrowth and scarring of various tissues characterized by the accumulation of extracellular matrix components. Aging is an important predisposing factor common for fibrotic heart and respiratory disease. Read More

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http://www.aginganddisease.org/EN/10.14336/AD.2018.0601
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http://dx.doi.org/10.14336/AD.2018.0601DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6457057PMC
April 2019
2 Reads

Multipotent Mesenchymal Stromal Cells for Pulmonary Fibrosis?

Am J Med Sci 2019 May 13;357(5):390-393. Epub 2019 Feb 13.

Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Miami Miller School of Medicine, Miami, Florida. Electronic address:

With the combination of ideologic beliefs and the will to survive, fraught patients and determined clinicians seek alternative therapies for treatment of terminal conditions, such as idiopathic pulmonary fibrosis. Unfortunately, the need for treatment has supported the growth of unapproved stem cell therapy over the years spanning across many countries. The reality, however, is that the science behind this therapy is lagging. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00029629193006
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http://dx.doi.org/10.1016/j.amjms.2019.02.007DOI Listing
May 2019
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The Aging Lung and Idiopathic Pulmonary Fibrosis.

Am J Med Sci 2019 May 12;357(5):384-389. Epub 2019 Feb 12.

Division of Pulmonary, Allergy, and Critical Care Medicine and; Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama.

Idiopathic pulmonary fibrosis (IPF) is one of many clinical syndromes that are associated with aging, and is increasing in both incidence and prevalence with the rapid rise in aging populations world-wide. There is accumulating data on how the biology of aging may influence the susceptibility to lung fibrosis in the elderly. In this review, we explore some of the known "hallmarks of aging," including telomere attrition, genomic instability, epigenetic alterations, loss of proteostasis, cellular senescence and mitochondrial dysfunction in the pathobiology of IPF. Read More

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http://dx.doi.org/10.1016/j.amjms.2019.02.008DOI Listing

Genetics of Idiopathic Pulmonary Fibrosis.

Am J Med Sci 2019 May 13;357(5):379-383. Epub 2019 Feb 13.

Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Virginia Health System, Charlottesville, Virginia. Electronic address:

Idiopathic pulmonary fibrosis (IPF) is the most common form of interstitial lung disease. IPF is a complex disease, with environmental and genetic factors variably contributing to disease susceptibility and outcomes. A host of common gene variants with modest effect size impart disease risk in patients with sporadic IPF, while rare variants with large effect size influence disease risk in those with familial interstitial pneumonia. Read More

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http://dx.doi.org/10.1016/j.amjms.2019.02.009DOI Listing

Epithelial Injury and Dysfunction in the Pathogenesis of Idiopathic PulmonaryFibrosis.

Am J Med Sci 2019 May 22;357(5):374-378. Epub 2019 Jan 22.

Department of Medicine, Division of Allergy, Pulmonary and Critical Care Medicineand; Department of Cell and Developmental Biology, Vanderbilt University, Nashville, Tennessee; Department of Veterans Affairs Medical Center, Nashville, Tennessee. Electronic address:

Idiopathic pulmonary fibrosis is a disease of older adults leading to progressive dyspnea and reduced exercise capacity, typically resulting in death within 3-5years of diagnosis. Underlying genetic susceptibility combined with environmental insults is proposed to trigger a chronic wound repair response, leading to activation of the fibrotic cascade. Perturbations in several molecular pathways mediate vulnerability of the alveolar epithelium to injurious agents, including the unfolded protein response, autophagy, mitophagy, and cellular senescence. Read More

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http://dx.doi.org/10.1016/j.amjms.2019.01.010DOI Listing

Current and Future Idiopathic Pulmonary Fibrosis Therapy.

Am J Med Sci 2019 May 11;357(5):370-373. Epub 2019 Feb 11.

Fondazione Policlinico Universitario A. Gemelli IRCCS, Università Cattolica del Sacro Cuore, Rome, Italy.

The last years have led to advances in the therapeutic management of idiopathic pulmonary fibrosis (IPF), mainly through the discovery of new pathological pathways and drugs and better design of clinical trials. The objective of this review is both to describe the current therapies approved for the treatment of IPF and the emerging therapeutic approaches. Currently, nintedanib and pirfenidone are the basis of IPF therapy, based on the results of large randomized clinical trials showing their safety and efficacy in reducing disease progression. Read More

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http://dx.doi.org/10.1016/j.amjms.2019.02.006DOI Listing

Idiopathic Pulmonary Fibrosis: Epidemiology, Diagnosis andOutcomes.

Am J Med Sci 2019 May 15;357(5):359-369. Epub 2019 Feb 15.

Department of Medicine, National Jewish Health, Denver, Colorado.

The incidence and prevalence of idiopathic pulmonary fibrosis (IPF) is increasing worldwide. This, combined with its poor prognosis and unpredictable natural history, has amplified the importance of an accurate diagnosis and monitoring. A diagnosis of exclusion, IPF requires a comprehensive clinical evaluation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00029629193007
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http://dx.doi.org/10.1016/j.amjms.2019.02.013DOI Listing
May 2019
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[Selection of lung transplant candidates in France in 2019].

Rev Mal Respir 2019 Apr 18. Epub 2019 Apr 18.

Pôle thorax et vaisseaux, clinique universitaire de pneumologie, CHU de Grenoble-Alpes, 38000 Grenoble, France; Université Grenoble-Alpes, 38000 Grenoble, France. Electronic address:

Introduction: In 2015, the International Society for Heart and Lung Transplantation (ISHLT) published a consensus document for the selection of lung transplant candidates. In the absence of recent French recommendations, this guideline is useful in order to send lung transplant candidates to the transplantation centers and to list them for lung transplantation at the right time.

Background: The main indications for lung transplantation in adults are COPD and emphysema, idiopathic pulmonary fibrosis and interstitial diseases, cystic fibrosis and pulmonary arterial hypertension (PAH). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S07618425193007
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http://dx.doi.org/10.1016/j.rmr.2018.03.009DOI Listing
April 2019
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The identification of CCL18 as biomarker of disease activity in localized scleroderma.

J Autoimmun 2019 Apr 18. Epub 2019 Apr 18.

Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, Utrecht, the Netherlands; Laboratory of Translational Immunology, University Medical Centre Utrecht, Utrecht, the Netherlands.

Background: Localized Scleroderma (LoS) encompasses a group of idiopathic skin conditions characterized by (sub)cutaneous inflammation and subsequent development of fibrosis. Currently, lack of accurate tools enabling disease activity assessment leads to suboptimal treatment approaches.

Objective: To investigate serum concentrations of cytokines and chemokines implicated in inflammation and angiogenesis in LoS and explore their potential to be utilized as biomarker of disease activity. Read More

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http://dx.doi.org/10.1016/j.jaut.2019.04.008DOI Listing

Characteristics and association with survival of respiratory-related hospitalization in Japanese idiopathic pulmonary fibrosis patients.

Respir Investig 2019 Apr 16. Epub 2019 Apr 16.

Department of Respiratory Medicine and Allergology, Kindai University, Faculty of Medicine, Osakasayama, Osaka, Japan. Electronic address:

Background: The characteristics and significance of respiratory-related hospitalization in patients with idiopathic pulmonary fibrosis (IPF) in Asian countries remain unknown. The purpose of this study was to define the characteristics of respiratory-related hospitalization and to inspect the relationship between respiratory-related hospitalization and subsequent survival in patients with IPF in Japanese general practice.

Methods: Patients with IPF who underwent clinical evaluation between February 2008 and August 2017 were screened. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22125345183031
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http://dx.doi.org/10.1016/j.resinv.2019.03.005DOI Listing
April 2019
5 Reads

Evaluation of changes in the serum levels of Krebs von den Lungen-6 and surfactant protein-D over time as important biomarkers in idiopathic fibrotic nonspecific interstitial pneumonia.

Respir Investig 2019 Apr 16. Epub 2019 Apr 16.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, 6-16-1 Tomioka-higashi, Kanazawa-ku, Yokohama 236-0051, Japan. Electronic address:

Background: Some cases of idiopathic fibrotic nonspecific interstitial pneumonia (f-NSIP) show a progressive course that is similar to that of idiopathic pulmonary fibrosis. However, it is difficult to predict poor patient outcomes. This study aimed to evaluate whether serial changes in serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) can predict disease progression. Read More

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http://dx.doi.org/10.1016/j.resinv.2019.03.006DOI Listing

Telomerase reverse transcriptase ameliorates lung fibrosis by protecting alveolar epithelial cells against senescence.

J Biol Chem 2019 Apr 18. Epub 2019 Apr 18.

Department of Pathology, University of Michigan.

Mutations in the genes encoding telomerase reverse transcriptase (TERT) and telomerase's RNA components, as well as shortened telomeres, are risk factors for idiopathic pulmonary fibrosis (IPF), wherein repetitive injury to the alveolar epithelium is considered to be a key factor in pathogenesis. Given the importance of TERT in stem cells, we hypothesized that TERT plays an important role in epithelial repair and that its deficiency results in exacerbation of fibrosis by impairing this repair/regenerative process. To evaluate the role of TERT in epithelial cells, we generated type II alveolar epithelial cell (AECII) specific TERT conditional knockout mice (SPC-Tert cKO) by crossing floxed TERT mice with inducible SPC-driven Cre mice. Read More

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http://dx.doi.org/10.1074/jbc.RA118.006615DOI Listing
April 2019
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Prognostic impact of the ratio of the main pulmonary artery to that of the aorta on chest computed tomography in patients with idiopathic pulmonary fibrosis.

BMC Pulm Med 2019 Apr 18;19(1):81. Epub 2019 Apr 18.

Division of Pulmonology, Department of Internal Medicine, Severance Hospital, Yonsei University College of Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul, 03722, Republic of Korea.

Background: In many clinical disorders, there is a relationship between the ratio of the diameter of the main pulmonary artery (mPA) to that of the aorta (Ao) on chest computed tomography (CT). The aim of this study was to determine if the mPA/Ao ratio at diagnosis is associated with the clinical characteristics and outcomes in patients with idiopathic pulmonary fibrosis (IPF).

Methods: We retrospectively reviewed the diameters of the pulmonary artery and aorta on chest CT, clinical characteristics, and results of other examinations in 303 patients at the time of initial diagnosis of IPF at our tertiary care center between 2011 and 2015. Read More

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https://bmcpulmmed.biomedcentral.com/articles/10.1186/s12890
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http://dx.doi.org/10.1186/s12890-019-0843-5DOI Listing
April 2019
4 Reads

Lipids - two sides of the same coin in lung fibrosis.

Cell Signal 2019 Apr 15;60:65-80. Epub 2019 Apr 15.

Department of Biochemistry, Universities of Giessen and Marburg Lung Center, Giessen, Germany. Electronic address:

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive extracellular matrix deposition in the lung parenchyma leading to the destruction of lung structure, respiratory failure and premature death. Recent studies revealed that the pathogenesis of IPF is associated with alterations in the synthesis and the activity of lipids, lipid regulating proteins and cell membrane lipid transporters and receptors in different lung cells. Furthermore, deregulated lipid metabolism was found to contribute to the profibrotic phenotypes of lung fibroblasts and alveolar epithelial cells. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08986568193008
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http://dx.doi.org/10.1016/j.cellsig.2019.04.007DOI Listing
April 2019
4 Reads

Predicting clinical outcome with phenotypic clusters using quantitative CT fibrosis and emphysema features in patients with idiopathic pulmonary fibrosis.

PLoS One 2019 18;14(4):e0215303. Epub 2019 Apr 18.

Division of Pulmonary and Critical Care Medicine, Department of Medicine, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.

Background: The clinical course of IPF varies. This study sought to identify phenotyping with quantitative computed tomography (CT) fibrosis and emphysema features using a cluster analysis and to assess prognostic impact among identified clusters in patient with idiopathic pulmonary fibrosis (IPF). Furthermore, we evaluated the impact of fibrosis and emphysema on lung function with development of a descriptive formula. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0215303PLOS
April 2019
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A robust data-driven genomic signature for idiopathic pulmonary fibrosis with applications for translational model selection.

PLoS One 2019 18;14(4):e0215565. Epub 2019 Apr 18.

Translational Bioinformatics, Translational Medicine, Bristol-Myers Squibb, Princeton, NJ, United States of America.

Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive lung disease affecting ~5 million people globally. We have constructed an accurate model of IPF disease status using elastic net regularized regression on clinical gene expression data. Leveraging whole transcriptome microarray data from 230 IPF and 89 control samples from Yang et al. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0215565PLOS

IL-25 contributes to lung fibrosis by directly acting on alveolar epithelial cells and fibroblasts.

Exp Biol Med (Maywood) 2019 Apr 18:1535370219843827. Epub 2019 Apr 18.

1 Department of Surgical Intensive Care Unit, Beijing An Zhen Hospital, Capital Medical University, Beijing 100029, China.

Impact Statement: Our work focused on alveolar epithelial cells (AECs)-derived type-2 cytokine (interleukin [IL]-25) in the pathogenesis of idiopathic pulmonary fibrosis (IPF). We showed that IL-25 and IL-17BR (IL-25's receptor) is upregulated in lung tissues (especially in AECs and lung fibroblasts) of IPF patients and contributes to lung fibrosis by directly activating lung fibroblasts and modulating epithelial-mesenchymal transition (EMT) of AECs. We suggest that IL-25 may be one of the master switches hidden in the milieu of abnormal epithelial-mesenchymal crosstalk. Read More

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http://dx.doi.org/10.1177/1535370219843827DOI Listing
April 2019
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Exploring the cross-phenotype network region of disease modules reveals concordant and discordant pathways between chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis.

Hum Mol Genet 2019 Apr 1. Epub 2019 Apr 1.

Channing Division of Network Medicine, Department of Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA.

Chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF) are two pathologically distinct chronic lung diseases that are associated with cigarette smoking. Genetic studies have identified shared loci for COPD and IPF, including several loci with opposite directions of effect. The existence of additional shared genetic loci, as well as potential shared pathobiological mechanisms between the two diseases at the molecular level, remains to be explored. Read More

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https://academic.oup.com/hmg/advance-article/doi/10.1093/hmg
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http://dx.doi.org/10.1093/hmg/ddz069DOI Listing
April 2019
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Obstructive sleep apnoea in patients with fibrotic diffuse parenchymal lung disease-characterization and treatment compliance assessment.

Can J Respir Ther 2018 1;54(2):35-40. Epub 2018 Aug 1.

Pulmonology Department, Centro Hospitalar São João, Oporto, Portugal.

Introduction: Recent studies have reported a high prevalence of obstructive sleep apnoea (OSA) among patients with diffuse parenchymal lung disease (DPLD), mainly idiopathic pulmonary fibrosis (IPF). Effective OSA treatment appears to have a positive impact on morbidity and mortality in these patients. However, poor compliance to positive airway pressure (PAP) treatment in fibrotic DPLD patients has been reported. Read More

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http://dx.doi.org/10.29390/cjrt-2018-005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6422053PMC
August 2018
1 Read

Genetic analyses of aplastic anemia and idiopathic pulmonary fibrosis patients with short telomeres, possible implication of DNA-repair genes.

Orphanet J Rare Dis 2019 Apr 17;14(1):82. Epub 2019 Apr 17.

Instituto de Investigaciones Biomedicas CSIC/UAM, IDIPaz, Arturo Duperier, 4, 28029, Madrid, Spain.

Background: Telomeres are nucleoprotein structures present at the terminal region of the chromosomes. Mutations in genes coding for proteins involved in telomere maintenance are causative of a number of disorders known as telomeropathies. The genetic origin of these diseases is heterogeneous and has not been determined for a significant proportion of patients. Read More

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https://ojrd.biomedcentral.com/articles/10.1186/s13023-019-1
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http://dx.doi.org/10.1186/s13023-019-1046-0DOI Listing
April 2019
4 Reads

Non-invasive Multi-modal Methods to Differentiate Inflamed vs Fibrotic Strictures in Patients With Crohn's Disease.

Clin Gastroenterol Hepatol 2019 Apr 14. Epub 2019 Apr 14.

IBD Centre, Humanitas Clinical and Research Centre, Rozzano, Milan, Italy; Department of Biomedical Sciences, Humanitas University, Rozzano, Milan, Italy. Electronic address:

Fibrotic strictures occur in 30% of patients with Crohn's disease (CD). However, there are no therapeutic agents that prevent or reverse fibrotic strictures. Strictures are treated by endoscopic dilatation procedures and surgical procedures, but there are high rates of recurrence. Read More

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http://dx.doi.org/10.1016/j.cgh.2019.04.025DOI Listing

Outcomes of Older Patients with Pulmonary Fibrosis and Non-Small Cell Lung Cancer.

Ann Am Thorac Soc 2019 Apr 16. Epub 2019 Apr 16.

Icahn School of Medicine at Mount Sinai, Medicine, New York, New York, United States.

Rationale: Characteristics and outcomes of lung cancer in patients with idiopathic pulmonary fibrosis (IPF) in the United States (US) remain understudied.

Objectives: To determine the tumor characteristics and survival of IPF patients with non-small cell lung cancer (NSCLC) using US population-based data.

Methods: We selected Medicare beneficiaries from the Surveillance, Epidemiology and End Results registry with histologically confirmed NSCLC diagnosed between 2007-2011. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201808-510OCDOI Listing
April 2019
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Long intergenic non-coding RNAs regulate human lung fibroblast function: Implications for idiopathic pulmonary fibrosis.

Sci Rep 2019 Apr 15;9(1):6020. Epub 2019 Apr 15.

Department of Pharmacy and Pharmacology, University of Bath, Claverton Down, Bath, BA2 7AY, United Kingdom.

Phenotypic changes in lung fibroblasts are believed to contribute to the development of Idiopathic Pulmonary Fibrosis (IPF), a progressive and fatal lung disease. Long intergenic non-coding RNAs (lincRNAs) have been identified as novel regulators of gene expression and protein activity. In non-stimulated cells, we observed reduced proliferation and inflammation but no difference in the fibrotic response of IPF fibroblasts. Read More

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http://www.nature.com/articles/s41598-019-42292-w
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http://dx.doi.org/10.1038/s41598-019-42292-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6465406PMC
April 2019
5 Reads

Knockdown of lncRNA H19 represses the progress of pulmonary fibrosis through the TGF-β/Smad3 pathway by regulating miR-140.

Mol Cell Biol 2019 Apr 15. Epub 2019 Apr 15.

Department of Respiration, The First Affiliated Hospital of Zhengzhou University, Zhengzhou.

Long non-coding RNAs (lncRNAs) are involved in various human diseases. Recently, H19 is reported to be upregulated in fibrotic rat lung and play a stimulative role in bleomycin (BLM)-induced pulmonary fibrosis in mice. However, its expression in human fibrotic lung tissues and action mechanism remain unclear. Read More

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http://dx.doi.org/10.1128/MCB.00143-19DOI Listing
April 2019
1 Read
4.777 Impact Factor

Diffuse Alveolar Hemorrhage Following Pirfenidone Initiation.

J Pharm Pract 2019 Apr 15:897190019841731. Epub 2019 Apr 15.

3 Pharmacy Practice and Medicine, Creighton University School of Pharmacy and Health Professions, Omaha, NE, USA.

Introduction: Diffuse alveolar hemorrhage (DAH) is bleeding into the alveolar space of the lungs. Pirfenidone is an antifibrotic agent that is approved for the treatment of idiopathic pulmonary fibrosis (IPF). The most commonly reported side effects include gastrointestinal and skin-related events. Read More

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http://dx.doi.org/10.1177/0897190019841731DOI Listing
April 2019
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Design of IPF Clinical Trials in the Era of Approved Therapies.

Am J Respir Crit Care Med 2019 Apr 15. Epub 2019 Apr 15.

Cornell Medical College, New York, New York, United States.

The approval of nintedanib and pirfenidone for treatment of idiopathic pulmonary fibrosis has introduced complexity into the design of clinical trials for new drugs on many levels. Placebo-controlled trials excluding background therapy may have issues with feasibility. Those that allow background therapy must consider the possibility of drug-drug interactions with existing therapies, as well as the narrower therapeutic window available to demonstrate incremental slowing of lung function decline with a new agent. Read More

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https://www.atsjournals.org/doi/10.1164/rccm.201903-0592PP
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http://dx.doi.org/10.1164/rccm.201903-0592PPDOI Listing
April 2019
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Combined Activation of Guanylate Cyclase and Cyclic AMP in Lung Fibroblasts as a Novel Therapeutic Concept for Lung Fibrosis.

Biomed Res Int 2019 7;2019:1345402. Epub 2019 Mar 7.

Pulmonary Cell Research, Department of Biomedicine and Pneumology, Department of Internal Medicine, University Hospital and University of Basel, Switzerland.

Remodelling of the peripheral lung tissue and fibrotic foci are the main pathologies of idiopathic pulmonary fibrosis (IPF), a disease that is difficult to treat. TGF- activation of peripheral lung fibroblasts is indicated as the major cause of tissue remodelling in IPF and is resulting in fibroblast hyperplasia and deposition of extracellular matrix. Soluble guanylate cyclase (sGC) stimulators combined with cyclic AMP (cAMP) activators have been reported to reduce proliferation and matrix deposition in other conditions than IPF. Read More

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https://www.hindawi.com/journals/bmri/2019/1345402/
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http://dx.doi.org/10.1155/2019/1345402DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431482PMC
March 2019
3 Reads

Pathogenesis of fibrostenosing Crohn's disease.

Transl Res 2019 Mar 26. Epub 2019 Mar 26.

Department of Inflammation and Immunity, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio; Department of Gastroenterology, Hepatology and Nutrition, Digestive Diseases and Surgery Institute, Cleveland Clinic Foundation, Cleveland, Ohio. Electronic address:

Crohn's disease (CD) is a chronic inflammatory disease, which could affect any part of the gastrointestinal tract. A severe complication of CD is fibrosis-associated strictures, which can cause bowel obstruction. Unfortunately, there is no specific antifibrotic therapy available. Read More

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http://dx.doi.org/10.1016/j.trsl.2019.03.005DOI Listing
March 2019
3 Reads

Criteria for the diagnosis of idiopathic pleuroparenchymal fibroelastosis: A proposal.

Respir Investig 2019 Apr 10. Epub 2019 Apr 10.

Department of Respiratory Medicine, Toho University Omori Medical Center, Japan.

Background: Physicians have few opportunities to perform surgical lung biopsy (SLB) to diagnose idiopathic pleuroparenchymal fibroelastosis (IPPFE). Therefore, diagnostic criteria for IPPFE that do not require SLB must be established. Herein, we propose diagnostic criteria for IPPFE with and without SLB. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22125345183025
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http://dx.doi.org/10.1016/j.resinv.2019.02.007DOI Listing
April 2019
6 Reads

Sarcoidosis and idiopathic pulmonary fibrosis: The same tale or a tale of two diseases in one.

Respir Med 2019 Apr 1. Epub 2019 Apr 1.

Center for Interstitial Lung Diseases, Division of Pulmonary and Critical Care and Sleep Medicine, University of Washington Medical Center, Seattle, WA, 98195, USA. Electronic address:

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http://dx.doi.org/10.1016/j.rmed.2019.03.021DOI Listing
April 2019
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Negative impact of anorexia and weight loss during prior pirfenidone administration on subsequent nintedanib treatment in patients with idiopathic pulmonary fibrosis.

BMC Pulm Med 2019 Apr 11;19(1):78. Epub 2019 Apr 11.

Department of Respiratory Medicine, Kanagawa Cardiovascular and Respiratory Center, Tomioka-Higashi 6-16-1, Kanazawa-ku, Yokohama, 236-0051, Japan.

Background: Current clinical practice guidelines for idiopathic pulmonary fibrosis (IPF) conditionally recommend use of pirfenidone and nintedanib. However, an optimal treatment sequence has not been established, and the data of treatment sequence from pirfenidone to nintedanib are limited. This study aimed to evaluate safety, tolerability and efficacy of nintedanib switched from pirfenidone in patients with IPF. Read More

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http://dx.doi.org/10.1186/s12890-019-0841-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458823PMC
April 2019
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Alteration of human macrophage phenotypes by the anti-fibrotic drug nintedanib.

Int Immunopharmacol 2019 Apr 8;72:112-123. Epub 2019 Apr 8.

Univ Rennes, Inserm, EHESP, Irset (Institut de recherche en santé, environnement et travail) - UMR_S 1085, F-35000 Rennes, France. Electronic address:

The tyrosine kinase inhibitor, Nintedanib (NTD), has been approved for the treatment of idiopathic pulmonary fibrosis (IPF). In cell-free systems, NTD was recently shown to inhibit kinase activity of the human recombinant colony-stimulating factor 1 (CSF1) receptor (CSF1R) which mediates major functions of pulmonary macrophages. In the present study, we have investigated the effects of NTD on the phenotype of human monocyte-derived macrophages controlled by CSF1 in order to identify its anti-inflammatory properties via CSF1R inhibition. Read More

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http://dx.doi.org/10.1016/j.intimp.2019.03.061DOI Listing
April 2019
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The Clinical Features of Bronchiectasis Associated with Alpha-1 Antitrypsin Deficiency, Common Variable Immunodeficiency and Primary Ciliary Dyskinesia--Results from the U.S. Bronchiectasis Research Registry.

Chronic Obstr Pulm Dis 2019 Apr 9;6(2). Epub 2019 Apr 9.

Oregon Health Sciences University Hospital, Portland.

Objective: This study compares and contrasts the clinical features of non-cystic fibrosis bronchiectasis with 3 uncommon disorders known to be associated with bronchiectasis but with distinctly different underlying defined pathophysiologic derangements, namely severe alpha-1 antitrypsin deficiency (AATD), common variable immunodeficiency (CVI) and primary ciliary dyskinesia (PCD).

Methods: The Bronchiectasis Research Registry provides a central database for studying patients with non-cystic fibrosis bronchiectasis. This report consists of information from 13 U. Read More

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http://dx.doi.org/10.15326/jcopdf.6.2.2018.0156DOI Listing
April 2019
8 Reads

The Mortality Burden of Idiopathic Pulmonary Fibrosis in the United Kingdom.

Am J Respir Crit Care Med 2019 Apr 11. Epub 2019 Apr 11.

Nottingham University, Nottingham, United Kingdom of Great Britain and Northern Ireland.

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http://dx.doi.org/10.1164/rccm.201902-0467LEDOI Listing
April 2019
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Glutamine Metabolism is Required for Collagen Protein Synthesis in Lung Fibroblasts.

Am J Respir Cell Mol Biol 2019 Apr 11. Epub 2019 Apr 11.

University of Chicago, 2462, Chicago, Illinois, United States ;

Idiopathic pulmonary fibrosis is characterized by the TGF-β-dependent differentiation of lung fibroblasts into myofibroblasts, leading to excessive deposition of extracellular matrix proteins, which distort lung architecture and function. Metabolic reprogramming in myofibroblasts is emerging as an important mechanism in the pathogenesis IPF and recent evidence suggests that glutamine metabolism is required in myofibroblasts although the exact role of glutamine in myofibroblasts is unclear. Here we demonstrate that glutamine, and its conversion to glutamate by glutaminase is required for TGF-β-induced collagen protein production in lung fibroblasts. Read More

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https://www.atsjournals.org/doi/10.1165/rcmb.2019-0008OC
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http://dx.doi.org/10.1165/rcmb.2019-0008OCDOI Listing
April 2019
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Long-term evaluation of home-based pulmonary rehabilitation in patients with fibrotic idiopathic interstitial pneumonias.

ERJ Open Res 2019 Apr 8;5(2). Epub 2019 Apr 8.

FormAction Santé, Pérenchies, France.

Background: Few studies have examined the benefits of pulmonary rehabilitation in patients with fibrotic idiopathic pulmonary pneumonia (f-IIP). Here, we report the results of an observational study in routine clinical practice of home-based pulmonary rehabilitation for f-IIP patients.

Methods: A total of 112 consecutive patients (61 with idiopathic pulmonary fibrosis and 51 with fibrotic nonspecific interstitial pneumonitis) were enrolled, of whom 65 had mild-to-moderate disease (forced vital capacity (FVC) ≥50% predicted and diffusing capacity of the lung for carbon monoxide () ≥30% predicted) and 47 had severe disease (FVC <50% predicted and/or <30% predicted). Read More

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http://dx.doi.org/10.1183/23120541.00045-2019DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452059PMC
April 2019
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AK106-001616, a potent and selective inhibitor of cytosolic phospholipase A2: in vivo efficacy for inflammation, neuropathic pain, and pulmonary fibrosis.

J Pharmacol Exp Ther 2019 Apr 10. Epub 2019 Apr 10.

Asahi Kasei Pharma Co. Ltd.

AK106-001616 is a novel, potent, and selective inhibitor of the cytosolic phospholipase A2 (cPLA2) enzyme. Unlike traditional non-steroidal anti-inflammatory drugs and selective cyclooxygenase-2 inhibitors, AK106-001616 reduced prostaglandin (PG) E2 and leukotriene (LT) B4 production by stimulated cells. The suppression of PGE2 and LTB4 production was also confirmed using an air pouch model in rats administered a single oral dose of AK106-001616. Read More

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http://dx.doi.org/10.1124/jpet.118.255034DOI Listing
April 2019
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Study protocol: exploring the efficacy of cyclophosphamide added to corticosteroids for treating acute exacerbation of idiopathic pulmonary fibrosis; a randomized double-blind, placebo-controlled, multi-center phase III trial (EXAFIP).

BMC Pulm Med 2019 Apr 11;19(1):75. Epub 2019 Apr 11.

Assistance Publique - Hôpitaux de Paris (AP-HP), Hôpital Avicenne, Service de pneumologie, Site constitutif du centre de référence des maladies pulmonaires rares OrphaLung, EA2363, Université Paris, 13, Bobigny, France.

Background: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease, with a median survival of 2-3 years and variable natural history, characterized by gradual and progressive deterioration. Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is a severe complication, associated with poor survival and a mortality > 50%. To date, no treatment has proven effective in AE-IPF, with cyclophosphamide (CYC) the only therapy suggested to be effective on survival, primarily based on retrospective series. Read More

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http://dx.doi.org/10.1186/s12890-019-0830-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458697PMC
April 2019
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Differing severities of acute exacerbations of idiopathic pulmonary fibrosis (IPF): insights from the INPULSIS® trials.

Respir Res 2019 Apr 11;20(1):71. Epub 2019 Apr 11.

Università Cattolica del Sacro Cuore, Fondazione Policlinico A. Gemelli, Rome, Italy.

Background: Given the broad definition of an acute exacerbation of IPF, it is likely that acute exacerbations are heterogeneous in their aetiology, severity and clinical course. We used pooled data from the INPULSIS® trials of nintedanib versus placebo to investigate whether acute exacerbations reported as serious adverse events were associated with higher mortality than those reported as non-serious adverse events and to assess the effect of nintedanib on these types of events.

Methods: Adverse events considered by an investigator to be an acute exacerbation were adjudicated as a confirmed acute exacerbation, suspected acute exacerbation, or not an acute exacerbation. Read More

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http://dx.doi.org/10.1186/s12931-019-1037-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6458653PMC
April 2019
1 Read