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Idiopathic pulmonary fibrosis

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https://www.readbyqxmd.com/read/29225786/recent-advances-in-managing-idiopathic-pulmonary-fibrosis
#1
REVIEW
Chiara Scelfo, Antonella Caminati, Sergio Harari
Idiopathic pulmonary fibrosis (IPF) is a rare pulmonary disease with a poor prognosis and severe impact on quality of life. Early diagnosis is still challenging and important delays are registered before final diagnosis can be reached. Available tools fail to predict the variable course of the disease and to evaluate response to antifibrotic drugs. Despite the recent approval of pirfenidone and nintedanib, significant challenges remain to improve prognosis and quality of life. It is hoped that the new insights gained in pathobiology in the last few years will lead to further advances in the diagnosis and management of IPF...
2017: F1000Research
https://www.readbyqxmd.com/read/29223018/targeting-oxidant-dependent-mechanisms-for-the-treatment-of-respiratory-diseases-and-their-comorbidities
#2
REVIEW
Neil C Thomson
Oxidative stress is implicated in the pathogenesis of respiratory diseases, such as COPD and its comorbidities, asthma, idiopathic pulmonary fibrosis and radiation pneumonitis. Antioxidants drugs, such as small molecule thiols, nuclear erythroid-2 related factor 2 activators and catalytic enzyme mimetics have been developed to target oxidant-dependent mechanisms. The therapeutic effects of antioxidants have been generally disappointing. A small number of antioxidants are approved for clinical use, such as the small molecule thiol N-acetyl-l-cysteine for chronic obstructive pulmonary disease, and in the United States, the superoxide dismutase mimetic AEOL 10150 for severe radiation pneumonitis...
December 6, 2017: Current Opinion in Pharmacology
https://www.readbyqxmd.com/read/29222007/current-concepts-in-pathogenesis-diagnosis-and-management-of-smoking-related-interstitial-lung-diseases
#3
REVIEW
Anupam Kumar, Sujith V Cherian, Robert Vassallo, Eunhee S Yi, Jay H Ryu
Tobacco exposure results in various changes to the airways and lung parenchyma. While emphysema represents the more common injury pattern, in some individuals, cigarette smoke injures alveolar epithelial and other lung cells resulting in diffuse infiltrates and parenchymal fibrosis. Smoking can trigger interstitial injury patterns mediated via recruitment and inappropriate persistence of myeloid and other immune cells including eosinophils. As our understanding of the role of cigarette smoke constituents in triggering lung injury continues to evolve, so does our recognition of the spectrum of smoking-related interstitial lung changes...
December 5, 2017: Chest
https://www.readbyqxmd.com/read/29221293/distance-saturation-product-of-the-6-minute-walk-test-predicts-mortality-of-patients-with-non-cystic-fibrosis-bronchiectasis
#4
Meng-Heng Hsieh, Yueh-Fu Fang, Fu-Tsai Chung, Chung-Shu Lee, Yu-Chen Chang, Yuan-Zhang Liu, Cheng-Hsien Wu, Horng-Chyuan Lin
Background: Previous surveillance methods to monitor the prognoses of patients with bronchiectasis are too complex for use in daily practice. The 6-minute walk test (6MWT) is a simple exercise test to predict the prognosis of chronic obstructive airway disease and numerous chronic lung diseases, including idiopathic pulmonary fibrosis. No studies have investigated exercise-induced oxygen desaturation (EID) and distance-saturation product (DSP) of 6MWT to predict the prognoses of patients with bronchiectasis...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29219887/short-term-automated-quantification-of-radiologic-changes-in-the-characterization-of-idiopathic-pulmonary-fibrosis-versus-nonspecific-interstitial-pneumonia-and-prediction-of-long-term-survival
#5
Federica De Giacomi, Sushravya Raghunath, Ronald Karwoski, Brian J Bartholmai, Teng Moua
PURPOSE: Fibrotic interstitial lung diseases presenting with nonspecific and overlapping radiologic findings may be difficult to diagnose without surgical biopsy. We hypothesized that baseline quantifiable radiologic features and their short-term interval change may be predictive of underlying histologic diagnosis as well as long-term survival in idiopathic pulmonary fibrosis (IPF) presenting without honeycombing versus nonspecific interstitial pneumonia (NSIP). MATERIALS AND METHODS: Forty biopsy-confirmed IPF and 20 biopsy-confirmed NSIP patients with available high-resolution chest computed tomography 4 to 24 months apart were studied...
December 6, 2017: Journal of Thoracic Imaging
https://www.readbyqxmd.com/read/29217661/foxo3-an-important-player-in-fibrogenesis-and-therapeutic-target-for-idiopathic-pulmonary-fibrosis
#6
Hamza M Al-Tamari, Swati Dabral, Anja Schmall, Pouya Sarvari, Clemens Ruppert, Jihye Paik, Ronald A DePinho, Friedrich Grimminger, Oliver Eickelberg, Andreas Guenther, Werner Seeger, Rajkumar Savai, Soni S Pullamsetti
Idiopathic pulmonary fibrosis (IPF) is a progressive and fatal parenchymal lung disease with limited therapeutic options, with fibroblast-to-myofibroblast transdifferentiation and hyperproliferation playing a major role. Investigating ex vivo-cultured (myo)fibroblasts from human IPF lungs as well as fibroblasts isolated from bleomycin-challenged mice, Forkhead box O3 (FoxO3) transcription factor was found to be less expressed, hyperphosphorylated, and nuclear-excluded relative to non-diseased controls. Downregulation and/or hyperphosphorylation of FoxO3 was reproduced by exposure of normal human lung fibroblasts to various pro-fibrotic growth factors and cytokines (FCS, PDGF, IGF1, TGF-β1)...
December 7, 2017: EMBO Molecular Medicine
https://www.readbyqxmd.com/read/29214586/epidemiology-of-rare-lung-diseases-the-challenges-and-opportunities-to-improve-research-and-knowledge
#7
Cormac McCarthy, Beatriz Lara Gallego, Bruce C Trapnell, Francis X McCormack
Rare lung diseases encompass a broad spectrum of conditions and affect an estimated 1.2-2.5 million people in North America and 1.5-3 million people in Europe. While individual rare lung diseases affect less than 1 in 2000 individuals, collectively they have a significant impact upon the population at large. Hence it is vital to understand firstly the epidemiology and subsequently the pathogenesis and clinical course of these disorders. Through a greater understanding of these aspects of disease, progress can be made in reducing symptoms, containing healthcare costs and utilizing resources efficiently...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/29214157/towards-a-global-initiative-for-fibrosis-treatment-gift
#8
Maria Molina-Molina, Alvar Agusti, Bruno Crestani, David A Schwartz, Melanie Königshoff, Rachel C Chambers, Toby M Maher, Rosa Faner, Ana Lucia Mora, Mauricio Rojas, Katerina M Antoniou, Jacobo Sellares
Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease characterised by increased scarring of lung tissue. Despite the recent introduction of novel drugs that slow disease progression, IPF remains a deadly disease, and the benefits of these new drugs differ markedly between patients. Human diseases arise due to alterations in an almost limitless network of interconnected genes, proteins, metabolites, cells and tissues, in direct relationship with a continuously changing macro- or microenvironment...
October 2017: ERJ Open Research
https://www.readbyqxmd.com/read/29212838/long-term-macrolides-in-diffuse-interstitial-lung-diseases
#9
REVIEW
Paola Faverio, Francesco Bini, Adriano Vaghi, Alberto Pesci
In the present review we provide currently available evidence for the use of macrolides in the treatment of diffuse interstitial lung diseases (ILDs). Up to now, research on macrolides has mainly focused on three areas. First, macrolides have shown some promising results in cellular models and case reports as antifibrotic agents, by promoting autophagy and clearance of intracellular protein aggregates and acting as regulators of surfactant homeostasis. Secondly, macrolides have an immunomodulatory effect, which has been applied in some organising pneumonia cases...
December 31, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29212837/pirfenidone-safety-and-adverse-event-management-in-idiopathic-pulmonary-fibrosis
#10
REVIEW
Lisa H Lancaster, Joao A de Andrade, Joseph D Zibrak, Maria L Padilla, Carlo Albera, Steven D Nathan, Marlies S Wijsenbeek, John L Stauffer, Klaus-Uwe Kirchgaessler, Ulrich Costabel
Pirfenidone is one of two approved therapies for the treatment of idiopathic pulmonary fibrosis (IPF). Randomised controlled clinical trials and subsequent post hoc analyses have demonstrated that pirfenidone reduces lung function decline, decreases mortality and improves progression-free survival. Long-term extension trials, registries and real-world studies have also shown similar treatment effects with pirfenidone. However, for patients with IPF to obtain the maximum benefits of pirfenidone treatment, the potential adverse events (AEs) associated with pirfenidone need to be managed...
December 31, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/29212488/identification-of-the-lipid-biomarkers-from-plasma-in-idiopathic-pulmonary-fibrosis-by-lipidomics
#11
Feng Yan, Zhensong Wen, Rui Wang, Wenling Luo, Yufeng Du, Wenjun Wang, Xianyang Chen
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is an irreversible interstitial pulmonary disease featured by high mortality, chronic and progressive course, and poor prognosis with unclear etiology. Currently, more studies have been focusing on identifying biomarkers to predict the progression of IPF, such as genes, proteins, and lipids. Lipids comprise diverse classes of molecules and play a critical role in cellular energy storage, structure, and signaling. The role of lipids in respiratory diseases, including cystic fibrosis, asthma and chronic obstructive pulmonary disease (COPD) has been investigated intensely in the recent years...
December 6, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29209910/efficacy-and-safety-of-nintedanib-for-the-treatment-of-idiopathic-pulmonary-fibrosis-an-update
#12
José Antonio Rodríguez-Portal
Idiopathic pulmonary fibrosis is a fatal form of progressive fibrosing interstitial pneumonia with limited treatment options. In recent years, its management has been transformed with the approval of two new antifibrotic drugs: nintedanib and pirfenidone. Nintedanib is a tyrosine kinase inhibitor that efficiently slows idiopathic pulmonary fibrosis progression and has an acceptable tolerability profile. This article reviews new available evidence on the long-term efficacy and safety of nintedanib in patients with idiopathic pulmonary fibrosis...
December 5, 2017: Drugs in R&D
https://www.readbyqxmd.com/read/29207437/unfavourable-outcome-of-glucocorticoid-treatment-in-suspected-idiopathic-pulmonary-fibrosis
#13
Ivo A Wiertz, Wim A Wuyts, Coline H M van Moorsel, Adriane D M Vorselaars, Hendrik W van Es, Matthijs F M van Oosterhout, Jan C Grutters
BACKGROUND AND OBJECTIVE: The diagnostic classification of 'possible idiopathic pulmonary fibrosis (posIPF)' is characterized by a radiological pattern of inconsistent usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT) scan and a UIP pattern in surgical lung biopsy (SLB). The evidence base to guide treatment for patients with posIPF is lacking; the clinician must choose between observation, treatment with immunomodulatory agents or anti-fibrotic agents. METHODS: To evaluate outcomes of immunomodulatory treatment, a multicentre cohort of 59 posIPF patients treated with prednisone was analysed retrospectively...
December 5, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/29206633/exploration-of-the-muc5b-promoter-variant-and-ild-risk-in-patients-with-autoimmune-myositis
#14
Cheilonda Johnson, Paul Rosen, Thomas Lloyd, Maureen Horton, Lisa Christopher-Stine, Chester V Oddis, Andrew L Mammen, Sonye K Danoff
Interstitial lung disease (ILD) is common in patients with autoimmune myositis but factors that determine susceptibility are unknown. Familial and sporadic idiopathic pulmonary fibrosis (IPF) are strongly associated with a single nucleotide polymorphism in the promoter region of MUC5B (rs35705950). We sought to determine the relationship between MUC5B polymorphism expression and myositis-ILD. The MUC5B minor allele frequency (MAF) was examined in 402 European American participants; 60 with idiopathic interstitial pneumonia (IIP), 208 with myositis-ILD, and 134 unaffected controls...
September 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29206498/the-potential-application-of-strategic-released-apigenin-from-polymeric-carrier-in-pulmonary-fibrosis
#15
Junxia Zhang, Liqin Chao, Xianghua Liu, Yanmei Shi, Caili Zhang, Lingfei Kong, Ruiqin Li
AIM: The capability of reducing fibrotic and inflammatory responses in lung tissues represents a gold standard for evaluating the efficacy of therapeutic interventions for treating idiopathic pulmonary fibrosis (IPF). A wide variety of therapeutic strategies have been employed in clinic to treat PF, but limited success has been obtained. Apigenin (4, 5, 7-trihydroxyflavone) is a member of flavonoid family that exerts anti-inflammatory and anti-fibrosis effects. In this study, we explore the potential therapeutic effect of apigenin in lung fibrosis...
December 5, 2017: Experimental Lung Research
https://www.readbyqxmd.com/read/29203758/-diffuse-idiopathic-pulmonary-neuroendocrine-cell-hyperplasia-dipnech-an-overview-of-the-cases-diagnosed-at-the-department-of-thoracic-surgery-in-the-years-2010-2014
#16
Mariusz Stefański, Krzysztof Bruliński, Marianna Stefańska
INTRODUCTION: Pulmonary neuroendocrine cells (PNEC) are present in the normal lungs with the incidence of 1 in 2500 epithelial cells. They usually proliferate in the presence of reactive processes related to inflammation and fibrosis of the lung parenchyma. The division of pulmonary neuroendocrine cell hyperplasia proposed by Travis et al. additionally distinguished diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) or proliferation that occurs in people without reactive hyperplasia risk factors...
2017: Wiadomości Lekarskie: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/29202450/angiotensin-converting-enzyme-defines-matrikine-regulated-inflammation-and-fibrosis
#17
Philip J O'Reilly, Qiang Ding, Samia Akthar, Guoqiang Cai, Kristopher R Genschmer, Dhiren F Patel, Patricia L Jackson, Liliana Viera, Mojtaba Roda, Morgan L Locy, Ellen A Bernstein, Clare M Lloyd, Kenneth E Bernstein, Robert J Snelgrove, J Edwin Blalock
The neutrophil chemoattractant proline-glycine-proline (PGP) is generated from collagen by matrix metalloproteinase-8/9 (MMP-8/9) and prolyl endopeptidase (PE), and it is concomitantly degraded by extracellular leukotriene A4 hydrolase (LTA4H) to limit neutrophilia. Components of cigarette smoke can acetylate PGP, yielding a species (AcPGP) that is resistant to LTA4H-mediated degradation and can, thus, support a sustained neutrophilia. In this study, we sought to elucidate if an antiinflammatory system existed to degrade AcPGP that is analogous to the PGP-LTA4H axis...
November 16, 2017: JCI Insight
https://www.readbyqxmd.com/read/29200204/thyroid-hormone-inhibits-lung-fibrosis-in-mice-by-improving-epithelial-mitochondrial-function
#18
Guoying Yu, Argyris Tzouvelekis, Rong Wang, Jose D Herazo-Maya, Gabriel H Ibarra, Anup Srivastava, Joao Pedro Werneck de Castro, Giuseppe DeIuliis, Farida Ahangari, Tony Woolard, Nachelle Aurelien, Rafael Arrojo E Drigo, Ye Gan, Morven Graham, Xinran Liu, Robert J Homer, Thomas S Scanlan, Praveen Mannam, Patty J Lee, Erica L Herzog, Antonio C Bianco, Naftali Kaminski
Thyroid hormone (TH) is critical for the maintenance of cellular homeostasis during stress responses, but its role in lung fibrosis is unknown. Here we found that the activity and expression of iodothyronine deiodinase 2 (DIO2), an enzyme that activates TH, were higher in lungs from patients with idiopathic pulmonary fibrosis than in control individuals and were correlated with disease severity. We also found that Dio2-knockout mice exhibited enhanced bleomycin-induced lung fibrosis. Aerosolized TH delivery increased survival and resolved fibrosis in two models of pulmonary fibrosis in mice (intratracheal bleomycin and inducible TGF-β1)...
December 4, 2017: Nature Medicine
https://www.readbyqxmd.com/read/29197520/effects-of-pirfenidone-in-acute-and-sub-chronic-liver-fibrosis-and-an-initiation-promotion-cancer-model-in-the-mouse
#19
Oleksii Seniutkin, Shinji Furuya, Yu-Syuan Luo, Joseph A Cichocki, Hisataka Fukushima, Yuki Kato, Hiromi Sugimoto, Tomoko Matsumoto, Takeki Uehara, Ivan Rusyn
Liver fibrosis results from chronic tissue damage and excessive regeneration with accumulation of extracellular matrix proteins; it is a precursor of liver cirrhosis and hepatocellular carcinoma. Liver fibrosis treatments are primarily directed at inflammation, with few options to combat fibrogenesis. Pirfenidone is a drug approved for idiopathic pulmonary fibrosis and this study was focused on anti-fibrotic and anti-cancer potential of pirfenidone in the liver of male B6C3F1/J mice. In a dose-finding study, mice were treated with CCl4 (0...
November 29, 2017: Toxicology and Applied Pharmacology
https://www.readbyqxmd.com/read/29197377/regulation-of-terra-on-telomeric-and-mitochondrial-functions-in-ipf-pathogenesis
#20
Yulin Gao, Jinjin Zhang, Yuxia Liu, Songzi Zhang, Youlei Wang, Bo Liu, Huizhu Liu, Rongrong Li, Changjun Lv, Xiaodong Song
BACKGROUND: Aging is a known risk factor of idiopathic pulmonary fibrosis (IPF). However, the pathogenic mechanisms underlying the effects of advanced aging remain largely unknown. Telomeric repeat-containing RNA (TERRA) represents a type of long noncoding RNA. In this study, the regulatory roles of TERRA on human telomeres and mitochondria and IPF epithelial injury model were identified. METHODS: Blood samples were collected from patients with IPF (n = 24) and matched control individuals (n = 24)...
December 2, 2017: BMC Pulmonary Medicine
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