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Pulmonary fibrosis idiopathic

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https://www.readbyqxmd.com/read/28922731/shikonin-suppresses-pulmonary-fibroblasts-proliferation-and-activation-by-regulating-akt-and-p38-mapk-signaling-pathways
#1
Yunjuan Nie, Yaoyao Yang, Jian Zhang, Guowei Cai, Yanhua Chang, Gaoshang Chai, Chongyong Guo
Fibroblast is believed to be the primary effector in idiopathic pulmonary fibrosis (IPF), a progressive lung disorder characterized by aberrant tissue remodeling and the formation of fibroblastic foci. Due to the complicated etiology and mechanism, there are few effective drugs for this fatal disease. Shikonin (SHI), which is the major ingredient isolated from the plant Lithospermum Erythrorhizon, has long been used as traditional medicine for many diseases including inflammation and cancer. The roles of SHI in attenuating skin scar and renal fibrosis by reducing TGFβ1-stimulated fibroblast activation are also reported...
September 14, 2017: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28921394/combination-therapy-with-pirfenidone-plus-prednisolone-ameliorates-paraquat-induced-pulmonary-fibrosis
#2
Rokhsana Rasooli, Fatemeh Pourgholamhosein, Younes Kamali, Fatemeh Nabipour, Ali Mandegary
Pirfenidone is known to slow the decline in vital capacity and increase survival in idiopathic pulmonary fibrosis (IPF). Besides, administration of glucocorticoids, e.g., prednisolone has been the conventional strategy to the treatment of patients with this disease, although their efficacy is under debate. Since multiple coactivated pathways are involved in the pathogenesis of IPF, combination therapy is a foundation strategy to cover many more synergetic mechanisms and increase response. The aim of the present study was to compare the therapeutic efficacy of prednisolone plus pirfenidone with pirfenidone alone in PQ-induced lung fibrosis...
September 18, 2017: Inflammation
https://www.readbyqxmd.com/read/28918867/telomere-shortening-in-idiopathic-pulmonary-fibrosis
#3
María Molina-Molina, Lurdes Planas-Cerezales, Rosario Perona
No abstract text is available yet for this article.
September 14, 2017: Archivos de Bronconeumología
https://www.readbyqxmd.com/read/28918851/using-evidence-in-clinical-practice-a-dream-coming-true-in-idiopathic-pulmonary-fibrosis
#4
EDITORIAL
G Sgalla, L Richeldi
No abstract text is available yet for this article.
September 2017: Revista Portuguesa de Pneumologia
https://www.readbyqxmd.com/read/28918442/the-role-of-periostin-in-lung-fibrosis-and-airway-remodeling
#5
REVIEW
David N O'Dwyer, Bethany B Moore
Periostin is a protein that plays a key role in development and repair within the biological matrix of the lung. As a matricellular protein that does not contribute to extracellular matrix structure, periostin interacts with other extracellular matrix proteins to regulate the composition of the matrix in the lung and other organs. In this review, we discuss the studies exploring the role of periostin to date in chronic respiratory diseases, namely asthma and idiopathic pulmonary fibrosis. Asthma is a major health problem globally affecting millions of people worldwide with significant associated morbidity and mortality...
September 16, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28915889/anti-fibrotic-efficacy-of-nintedanib-in-pulmonary-fibrosis-via-the-inhibition-of-fibrocyte-activity
#6
Seidai Sato, Shintaro Shinohara, Shinya Hayashi, Shun Morizumi, Shuichi Abe, Hiroyasu Okazaki, Yanjuan Chen, Hisatsugu Goto, Yoshinori Aono, Hirohisa Ogawa, Kazuya Koyama, Haruka Nishimura, Hiroshi Kawano, Yuko Toyoda, Hisanori Uehara, Yasuhiko Nishioka
BACKGROUND: Nintedanib, a tyrosine kinase inhibitor that is specific for platelet-derived growth factor receptors (PDGFR), fibroblast growth factor receptors (FGFR), and vascular endothelial growth factor receptors (VEGFR), has recently been approved for idiopathic pulmonary fibrosis. Fibrocytes are bone marrow-derived progenitor cells that produce growth factors and contribute to fibrogenesis in the lungs. However, the effects of nintedanib on the functions of fibrocytes remain unclear...
September 15, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28915874/unmet-needs-in-the-treatment-of-idiopathic-pulmonary-fibrosis-insights-from-patient-chart-review-in-five-european-countries
#7
Toby M Maher, Maria Molina-Molina, Anne-Marie Russell, Francesco Bonella, Stéphane Jouneau, Elena Ripamonti, Judit Axmann, Carlo Vancheri
BACKGROUND: Two antifibrotic drugs, pirfenidone and nintedanib, are approved by the European Medicines Agency and the US Food and Drug Administration for the treatment of idiopathic pulmonary fibrosis (IPF). In this analysis, treatment patterns of European patients with IPF were investigated to understand antifibrotic prescribing and identify unmet needs in IPF treatment practice. METHODS: Between February and March 2016, respiratory physicians from France, Germany, Italy, Spain, and the UK participated in an online questionnaire designed to collect information on IPF treatment patterns in patients under their care...
September 15, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28898890/an-open-label-study-of-the-long-term-safety-of-pirfenidone-in-patients-with-idiopathic-pulmonary-fibrosis-recap
#8
Ulrich Costabel, Carlo Albera, Lisa H Lancaster, Chin-Yu Lin, Philip Hormel, Henry N Hulter, Paul W Noble
BACKGROUND: RECAP (NCT00662038) was an open-label extension study in patients with idiopathic pulmonary fibrosis (IPF) who completed either the Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis (ASCEND) 016 phase 3 trial or the Clinical Studies Assessing Pirfenidone in Idiopathic Pulmonary Fibrosis: Research of Efficacy and Safety Outcomes (CAPACITY) 004/006 phase 3 trials. OBJECTIVE: To obtain long-term safety data for pirfenidone in patients with IPF in RECAP...
September 13, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28892504/clinical-impact-of-depression-and-anxiety-in-patients-with-idiopathic-pulmonary-fibrosis
#9
Ye Jin Lee, Sun Mi Choi, Yeon Joo Lee, Young-Jae Cho, Ho Il Yoon, Jae-Ho Lee, Choon-Taek Lee, Jong Sun Park
BACKGROUND: Although depression and anxiety represent significant yet treatable comorbidities in patients with idiopathic pulmonary fibrosis (IPF), their impact on the clinical course and prognosis of IPF remain unclear. PURPOSE: We investigated the prevalence and clinical significance of depression and anxiety in patients with IPF. METHODS: The present study included a prospective cohort comprising 112 Korean patients with IPF who had completed the Hospital Anxiety and Depression Scale (HADS) questionnaire...
2017: PloS One
https://www.readbyqxmd.com/read/28892311/-how-i-treat-a-patient-with-idiopathic-pulmonary-fibrosis
#10
J Guiot, B Duysinx, O Bonhomme, R Louis, J-L Corhay
Idiopathic pulmonary fibrosis (IPF) is a rare disorder of unknown origin, which is associated with a high mortality and whose incidence has been increasing for several years. Nowadays there are two anti-fibrotic therapies (pirfenidone - nintedanib) known to reduce significantly the decline in respiratory function tests of patients suffering from this condition. The only curative therapeutic option remains the pulmonary transplantation whose accessibility remains limited. Pulmonary rehabilitation is also central in the treatment of patients...
September 2017: Revue Médicale de Liège
https://www.readbyqxmd.com/read/28889759/nintedanib-with-add-on-pirfenidone-in-idiopathic-pulmonary-fibrosis-results-of-the-injourney-trial
#11
Carlo Vancheri, Michael Kreuter, Luca Richeldi, Christopher J Ryerson, Dominique Valeyre, Jan C Grutters, Sabrina Wiebe, Wibke Stansen, Manuel Quaresma, Susanne Stowasser, Wim A Wuyts
RATIONALE: Nintedanib and pirfenidone slow the progression of IPF, but the disease continues to progress. More data are needed on the safety and efficacy of combination therapy with nintedanib and add-on pirfenidone. OBJECTIVES: To investigate safety, tolerability, pharmacokinetic and exploratory efficacy endpoints in patients treated with nintedanib and add-on pirfenidone versus nintedanib alone. METHODS: Patients with IPF and FVC ≥50% predicted at screening who completed a 4-5 week run-in with nintedanib 150 mg bid without dose reduction or treatment interruption were randomized to nintedanib 150 mg bid with add-on pirfenidone (titrated to 801 mg tid), or nintedanib 150 mg bid alone, open-label for 12 weeks...
September 10, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/28889145/efficacy-and-adverse-events-of-pirfenidone-in-treating-idiopathic-pulmonary-fibrosis
#12
Hui Ren, Kai Wang, Hao Yang, Lingyun Gao
To analyze the efficacy and adverse events  (AEs) of pirfenidone in idiopathic pulmonary fibrosis (IPF) trials. Methods: MEDLINE, Cochrane Library, and ClinicalTrials.gov were searched for studies published before June 2016. All studies of clinical trials with the key words IPF or idiopathic pulmonary fibrosis or lung fibrosis and pirfenidone or Esbriet were identified. Quality assessment and data extraction nwere conducted by 2 independent researchers. A meta-analysis of randomized controlled trials (RCTs) was performed, and relative risk (RR) and 95% confidence intervals (95% CIs) were calculated...
September 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/28886713/increased-activated-regulatory-t-cells-proportion-correlate-with-the-severity-of-idiopathic-pulmonary-fibrosis
#13
Ziliang Hou, Qiao Ye, Meihua Qiu, Yu Hao, Junyan Han, Hui Zeng
BACKGROUND: Regulatory T cells (Tregs) are crucial in maintaining immune tolerance and immune homeostasis, but their role in idiopathic pulmonary fibrosis (IPF) is unclear. This study was designed to explore the role of Tregs in IPF. METHODS: Percentages of Tregs and their subpopulations in peripheral blood (PB) and bronchoalveolar lavage (BAL) samples were determined by flow cytometry in 29 patients with IPF, 19 patients with primary Sjögren's syndrome-related interstitial pneumonia (pSS-IP), and 23 healthy controls (HCs)...
September 8, 2017: Respiratory Research
https://www.readbyqxmd.com/read/28886193/estimating-the-incidence-of-interstitial-lung-diseases-in-the-cree-of-eeyou-istchee-northern-qu%C3%A3-bec
#14
Matthieu Storme, Alexandre Semionov, Deborah Assayag, Michael Lefson, Darlene Kitty, David Dannenbaum, Jill Torrie, Pierre Lejeune, Elizabeth Robinson, Faiz Ahmad Khan
BACKGROUND: Little is known about the epidemiology of interstitial lung disease (ILD) amongst Canada's Indigenous populations. Clinicians working in Eeyou Istchee (the Cree territory of the James Bay region of Québec, population 17, 956) suspected that ILD was more common in this area. We sought to identify all prevalent and incident cases of ILD in Eeyou Istchee between 2006 and 2013, to describe characteristics of affected patients, distribution of subtypes, and estimate disease incidence...
2017: PloS One
https://www.readbyqxmd.com/read/28883926/clinical-use-of-nintedanib-in-patients-with-idiopathic-pulmonary-fibrosis
#15
REVIEW
Amy Hajari Case, Peace Johnson
Idiopathic pulmonary fibrosis (IPF) is a rare lung disease characterised by progressive loss of lung function, dyspnoea and cough. IPF has a variable clinical course but a poor prognosis. Nintedanib, a tyrosine kinase inhibitor, is one of two drugs approved for the treatment of IPF. In clinical trials, nintedanib slowed disease progression by reducing the rate of decline in forced vital capacity (FVC) in patients with IPF and mild or moderate lung function impairment. The effect of nintedanib was consistent across patient subgroups defined by baseline characteristics including FVC % predicted, diffusion capacity of the lung for carbon monoxide % predicted and the presence of emphysema...
2017: BMJ Open Respiratory Research
https://www.readbyqxmd.com/read/28883924/metabolic-heterogeneity-of-idiopathic-pulmonary-fibrosis-a-metabolomic-study
#16
Yidan D Zhao, Li Yin, Stephen Archer, Catherine Lu, George Zhao, Yan Yao, Licun Wu, Michael Hsin, Thomas K Waddell, Shaf Keshavjee, John Granton, Marc de Perrot
INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is a chronic and fatal disease of unknown cause characterised by progressive fibrotic formation in lung tissue. We hypothesise that disrupted metabolic pathways in IPF contribute to disease pathogenesis. METHODS: Metabolomics of human IPF was performed using mass spectroscopy (IPF lung=8; donor lung=8). Gene expression of key metabolic enzymes was measured using microarrays. Of the 108 metabolites whose levels were found altered, 48 were significantly increased, whereas 60 were significantly decreased in IPF samples compared with normal controls...
2017: BMJ Open Respiratory Research
https://www.readbyqxmd.com/read/28883482/low-body-surface-area-predicts-hepatotoxicity-of-nintedanib-in-patients-with-idiopathic-pulmonary-fibrosis
#17
Satoshi Ikeda, Akimasa Sekine, Tomohisa Baba, Yumie Yamanaka, Shinko Sadoyama, Hideaki Yamakawa, Tsuneyuki Oda, Ryo Okuda, Hideya Kitamura, Koji Okudela, Tae Iwasawa, Kenichi Ohashi, Tamiko Takemura, Takashi Ogura
After the commercialization of nintedanib in Japan, a high incidence of hepatotoxicity resulting in treatment interruption was noted in idiopathic pulmonary fibrosis (IPF) patients treated with nintedanib in our hospital. This study aimed to clarify the risk factors for hepatotoxicity of nintedanib. Sixty-eight consecutive cases of IPF newly treated with nintedanib at a dose of 150 mg twice daily from September 2015 to September 2016 were enrolled: 46 patients (67.6%) exhibited aspartate aminotransferase (AST) and/or alanine aminotransferase (ALT) elevation and 16 patients (23...
September 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28883090/validity-responsiveness-and-minimum-clinically-important-difference-of-the-incremental-shuttle-walk-in-idiopathic-pulmonary-fibrosis-a-prospective-study
#18
Claire M Nolan, Veronica Delogu, Matthew Maddocks, Suhani Patel, Ruth E Barker, Sarah E Jones, Samantha S C Kon, Toby M Maher, Paul Cullinan, William D-C Man
The incremental shuttle walk (ISW) is well validated in COPD but limited psychometric data restrict its use in idiopathic pulmonary fibrosis (IPF). Study 1: 50 patients performed the ISW and 6 min walk test (6MWT). Study 2: 72 patients completed the ISW before and after pulmonary rehabilitation (PR). The ISW correlated strongly with 6MWT distance (r=0.81,p<0.0001). Mean (95% confidence interval) improvement in ISW with PR was 54 (38 to 70) m with an effect size of 0.29. Distribution-based and anchor-based minimum clinically important difference (MCID) estimates ranged from 31 to 46 m...
September 7, 2017: Thorax
https://www.readbyqxmd.com/read/28879080/diffuse-parenchymal-lung-disease-with-micro-aspirations-in-presence-of-hiatal-hernia
#19
Rishitha Yelisetti, Areig Awad, Anand Kaji
There has been controversy regarding the relationship between gastroesophageal reflux, microaspiration, and idiopathic pulmonary fibrosis (IPF). In the last decade, there is increasing evidence supporting a relationship between gastroesophageal reflux, microaspiration, and IPF. The presence of hiatal hernia further propagates microaspirations. Surgical management of hiatal hernia plays a vital role in the treatment.
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28878780/p2y6-receptor-activation-promotes-inflammation-and-tissue-remodeling-in-pulmonary-fibrosis
#20
Tobias Müller, Susanne Fay, Rodolfo Paula Vieira, Harry Karmouty-Quintana, Sanja Cicko, Cemil Korcan Ayata, Gernot Zissel, Torsten Goldmann, Giuseppe Lungarella, Davide Ferrari, Francesco Di Virgilio, Bernard Robaye, Jean-Marie Boeynaems, Eduardo R Lazarowski, Michael R Blackburn, Marco Idzko
Idiopathic pulmonary fibrosis (IPF) is a disease with a poor prognosis and very few available treatment options. The involvement of the purinergic receptor subtypes P2Y2 and P2X7 in fibrotic lung disease has been demonstrated recently. In this study, we investigated the role of P2Y6 receptors in the pathogenesis of IPF in humans and in the animal model of bleomycin-induced lung injury. P2Y6R expression was upregulated in lung structural cells but not in bronchoalveolar lavage (BAL) cells derived from IPF patients as well as in animals following bleomycin administration...
2017: Frontiers in Immunology
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