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https://www.readbyqxmd.com/read/28993806/genetics-in-idiopathic-pulmonary-fibrosis-pathogenesis-prognosis-and-treatment
#1
REVIEW
Amarpreet Kaur, Susan K Mathai, David A Schwartz
Idiopathic pulmonary fibrosis (IPF), the most common form of idiopathic interstitial pneumonia (IIP), is characterized by irreversible scarring of the lung parenchyma and progressive decline in lung function leading to eventual respiratory failure. The prognosis of IPF is poor with a median survival of 3-5 years after diagnosis and no curative medical therapies. Although the pathogenesis of IPF is not well understood, there is a growing body of evidence that genetic factors contribute to disease risk. Recent studies have identified common and rare genetic variants associated with both sporadic and familial forms of pulmonary fibrosis, with at least one-third of the risk for developing fibrotic IIP explained by common genetic variants...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28981359/dendriform-pulmonary-ossification-in-the-absence-of-usual-interstitial-pneumonia-ct-features-and-possible-association-with-recurrent-acid-aspiration
#2
James F Gruden, Daniel B Green, Alan C Legasto, Eric A Jensen, Prasad M Panse
OBJECTIVE: Dendriform pulmonary ossification (DPO) is a rare lung disease in which mature bone is present in the peripheral interstitium of the lung. It typically occurs in patients with usual interstitial pneumonia (UIP). We assessed patients with CT findings of DPO without UIP to determine possible causative factors and to assess the clinical and CT course. We hypothesized that DPO without UIP would be a unique entity. MATERIALS AND METHODS: We retrospectively reviewed CT reports for the word "ossification...
October 5, 2017: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/28978213/evaluating-new-treatment-options
#3
Steven D Nathan
Idiopathic pulmonary fibrosis (IPF) is the most prevalent type of idiopathic interstitial pneumonia, accounting for at least half of all diagnosed cases. Because it lacks a cure, the goal of treatment for IPF is to stabilize or reduce the rate of disease progression. Nonpharmacologic treatment options for IPF consist of long-term oxygen treatment, lung transplantation, and pulmonary rehabilitation. In the past, pharmacologic therapies for IPF included anticoagulants and anti-inflammatory or immunosuppressive agents...
July 2017: American Journal of Managed Care
https://www.readbyqxmd.com/read/28978212/overview-of-idiopathic-pulmonary-fibrosis-ipf-and-evidence-based-guidelines
#4
Roozbeh Sharif
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive form of interstitial lung disease (ILD), characterized by fibrosis and worsening lung function, that primarily occurs in those 50 years and older. Various causes including genetic susceptibility, environmental risk factors, and exposures have been suggested in the literature. All of these cause repetitive micro-injury to the lung tissue and vasculature, which triggers a cascade of inflammatory response and fibrosis. Symptoms are nonspecific and most patients present several years after the initial radiographic changes occur...
July 2017: American Journal of Managed Care
https://www.readbyqxmd.com/read/28963678/serial-automated-quantitative-ct-analysis-in-idiopathic-pulmonary-fibrosis-functional-correlations-and-comparison-with-changes-in-visual-ct-scores
#5
Joseph Jacob, Brian J Bartholmai, Srinivasan Rajagopalan, Maria Kokosi, Ryoko Egashira, Anne Laure Brun, Arjun Nair, Simon L F Walsh, Ronald Karwoski, Athol U Wells
OBJECTIVES: To determine whether computer-based CT quantitation of change can improve on visual change quantification of parenchymal features in IPF. METHODS: Sixty-six IPF patients with serial CT imaging (6-24 months apart) had CT features scored visually and with a computer software tool: ground glass opacity, reticulation and honeycombing (all three variables summed as interstitial lung disease extent [ILD]) and emphysema. Pulmonary vessel volume (PVV) was estimated by computer only...
September 29, 2017: European Radiology
https://www.readbyqxmd.com/read/28957531/fibroblasts-and-the-extracellular-matrix-in-right-ventricular-disease
#6
Nikolaos G Frangogiannis
Right ventricular failure predicts adverse outcome in patients with pulmonary hypertension (PH), and in subjects with left ventricular heart failure and is associated with interstitial fibrosis. This review manuscript discusses the cellular effectors and molecular mechanisms implicated in right ventricular fibrosis. The right ventricular interstitium contains vascular cells, fibroblasts, and immune cells, enmeshed in a collagen-based matrix. Right ventricular pressure overload in PH is associated with the expansion of the fibroblast population, myofibroblast activation, and secretion of extracellular matrix proteins...
October 1, 2017: Cardiovascular Research
https://www.readbyqxmd.com/read/28954767/pulmonary-hypertension-in-systemic-sclerosis-different-phenotypes
#7
REVIEW
David Launay, Vincent Sobanski, Eric Hachulla, Marc Humbert
Pulmonary hypertension (PH) is a frequent and severe complication of systemic sclerosis (SSc). PH in SSc is highly heterogeneous because of the various clinical phenotypes of SSc itself and because the mechanisms of PH can vary from one patient to another. PH in SSc may be due to vasculopathy of the small pulmonary arteries (group 1; pulmonary arterial hypertension), interstitial lung disease (group 3; PH due to lung disease or chronic hypoxia) or myocardial fibrosis leading to left ventricular systolic or diastolic dysfunction (group 2; PH due to chronic left-heart disease)...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28954766/recent-lessons-learned-in-the-management-of-acute-exacerbation-of-idiopathic-pulmonary-fibrosis
#8
Yasuhiro Kondoh, Vincent Cottin, Kevin K Brown
Recognising recent advances, the definition and diagnostic criteria for acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) have been updated by an international working group. The new definition describes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events) characterised by evidence of new widespread alveolar abnormality. The new criteria require a previous or concurrent diagnosis of IPF, an acute worsening or development of dyspnoea typically less than 1 month in duration, chest imaging evidence on computed tomography (CT) of new bilateral ground-glass opacity and/or consolidation superimposed on a background imaging pattern of usual interstitial pneumonia not fully explained by cardiac failure or fluid overload...
September 30, 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28953449/hepatitis-c-virus-associated-extrahepatic-manifestations-in-lung-and-heart-and-antiviral-therapy-related-cardiopulmonary-toxicity
#9
Syeda Zainab Ilyas, Rabia Tabassum, Haroon Hamed, Shafiq Ur Rehman, Ishtiaq Qadri
Besides liver cirrhosis and hepatocellular carcinoma, chronic hepatitis C virus (HCV) infection is associated with many extrahepatic manifestations (EHMs). HCV exhibits lymphotropism that is responsible for various EHM. An important characteristic of HCV is escape from the immune system, which enables it to produce chronic infections and autoimmune disorders along with accumulation of circulating immune complexes. These EHMs have large spectrum, because they affect many organs such as heart, lungs, kidney, brain, thyroid, and skin...
September 27, 2017: Viral Immunology
https://www.readbyqxmd.com/read/28951138/curcumin-attenuates-skeletal-muscle-mitochondrial-impairment-in-copd-rats-pgc-1%C3%AE-sirt3-pathway-involved
#10
Ming Zhang, Jingjing Tang, Yali Li, Yingying Xie, Hu Shan, Mingxia Chen, Jie Zhang, Xia Yang, Qiuhong Zhang, Xudong Yang
Curcumin has been widely used to treat numerous diseases due to its antioxidant property. The aim of the present study is to investigate the effect of curcumin on skeletal muscle mitochondria in chronic obstructive pulmonary disease (COPD) and its underlying mechanism. The rat model of COPD was established by cigarette smoke exposure combined with intratracheal administration of lipopolysaccharide. Airway inflammation and emphysema were notably ameliorated by the treatment with curcumin. Oral administration of curcumin significantly improved muscle fiber atrophy, myofibril disorganization, interstitial fibrosis and mitochondrial structure damage in the skeletal muscle of COPD rats...
September 23, 2017: Chemico-biological Interactions
https://www.readbyqxmd.com/read/28947046/frailty-and-maximal-exercise-capacity-in-adult-lung-transplant-candidates
#11
Aimee M Layton, Hilary F Armstrong, Matthew R Baldwin, Anna J Podolanczuk, Nicole M Pieszchata, Jonathan P Singer, Selim M Arcasoy, Kimberly S Meza, Frank D'Ovidio, David J Lederer
BACKGROUND: Frail lung transplant candidates are more likely to be delisted or die without receiving a transplant. Further knowledge of what frailty represents in this population will assist in developing interventions to prevent frailty from developing. We set out to determine whether frail lung transplant candidates have reduced exercise capacity independent of disease severity and diagnosis. METHODS: Sixty-eight adult lung transplant candidates underwent cardiopulmonary exercise testing (CPET) and a frailty assessment (Fried's Frailty Phenotype (FFP))...
October 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28947042/therapeutic-targets-in-idiopathic-pulmonary-fibrosis
#12
REVIEW
Martin Kolb, Francesco Bonella, Lutz Wollin
Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal interstitial lung disease. After many drugs failed in clinical trials, improvements in the understanding of the pathogenesis of IPF led to the approval of two drugs that slow the progression of the disease. However, the prognosis for patients with IPF remains poor and the search continues for drugs that inhibit the pathogenic pathways active in IPF to reduce or even halt the progression of the disease. In this article, we review the mechanisms of action of the two approved therapies for IPF (nintedanib and pirfenidone) and of the investigational compounds that are in Phase II trials and discuss the potential for combination therapy in the treatment of IPF...
October 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28947036/possible-uip-pattern-on-high-resolution-computed-tomography-is-associated-with-better-survival-than-definite-uip-in-ipf-patients
#13
Margaret L Salisbury, Leslie B Tolle, Meng Xia, Susan Murray, Nabihah Tayob, Anoop M Nambiar, Shelley L Schmidt, Amir Lagstein, Jeffery L Myers, Barry H Gross, Ella A Kazerooni, Baskaran Sundaram, Aamer R Chughtai, Fernando J Martinez, Kevin R Flaherty
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrosing lung disease of unknown etiology. Inter-society consensus guidelines on IPF diagnosis and management outline radiologic patterns including definite usual interstitial pneumonia (UIP), possible UIP, and inconsistent with UIP. We evaluate these diagnostic categories as prognostic markers among patients with IPF. METHODS: Included subjects had biopsy-proven UIP, a multidisciplinary team diagnosis of IPF, and a baseline high-resolution computed tomography (HRCT)...
October 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/28942884/overexpression-of-il-38-protein-in-anticancer-drug-induced-lung-injury-and-acute-exacerbation-of-idiopathic-pulmonary-fibrosis
#14
Masaki Tominaga, Masaki Okamoto, Tomotaka Kawayama, Masanobu Matsuoka, Shinjiro Kaieda, Yuki Sakazaki, Takashi Kinoshita, Daisuke Mori, Akira Inoue, Tomoaki Hoshino
BACKGROUND: Interleukin (IL)-38, a member of the IL-1 family, shows high homology to IL-1 receptor antagonist (IL-1Ra) and IL-36 receptor antagonist (IL-36Ra). Its function in interstitial lung disease (ILD) is still unknown. METHODS: To determine the expression pattern of IL-38 mRNA, a panel of cDNAs derived from various tissues was analyzed by quantitative real-time PCR. Immunohistochemical reactivity with anti-human IL-38 monoclonal antibody (clone H127C) was evaluated semi-quantitatively in lung tissue samples from 12 patients with idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP), 5 with acute exacerbation of IPF, and 10 with anticancer drug-induced ILD (bleomycin in 5 and epidermal growth factor receptor-tyrosine kinase inhibitor in 5)...
September 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28936704/extraction-of-the-subpleural-lung-region-from-computed-tomography-images-to-detect-interstitial-lung-disease
#15
Tae Iwasawa, Yuma Iwao, Tamiko Takemura, Koji Okudela, Toshiyuki Gotoh, Tomohisa Baba, Takashi Ogura, Mari S Oba
PURPOSE: To quantify lesions in the subpleural lung region (SubPL) on computed tomography (CT) images and to evaluate whether they are useful for detecting interstitial lung disease (ILD). MATERIALS AND METHODS: The subjects were 40 patients with idiopathic pulmonary fibrosis (IPF) diagnosed by multidisciplinary methods and 35 age-matched patients without ILDs. The lungs and SubPL were extracted from CT images using a Gaussian histogram normalized correlation system and evaluated for the mean CT attenuation value (CTmean) and the percentage of high attenuation area (%HAA), exceeding -700 Hounsfield units...
September 21, 2017: Japanese Journal of Radiology
https://www.readbyqxmd.com/read/28934917/sparse-supervised-classification-methods-predict-and-characterize-nanomaterial-exposures-independent-markers-of-mwcnt-exposures
#16
Naveena Yanamala, Marlene S Orandle, Vamsi K Kodali, Lindsey Bishop, Patti C Zeidler-Erdely, Jenny R Roberts, Vincent Castranova, Aaron Erdely
Recent experimental evidence indicates significant pulmonary toxicity of multiwalled carbon nanotubes (MWCNTs), such as inflammation, interstitial fibrosis, granuloma formation, and carcinogenicity. Although numerous studies explored the adverse potential of various CNTs, their comparability is often limited. This is due to differences in administered dose, physicochemical characteristics, exposure methods, and end points monitored. Here, we addressed the problem through sparse classification method, a supervised machine learning approach that can reduce the noise contained in redundant variables for discriminating among MWCNT-exposed and MWCNT-unexposed groups...
January 1, 2017: Toxicologic Pathology
https://www.readbyqxmd.com/read/28932540/dynamic-expression-of-transformating-growth-factor-%C3%AE-1-and-caveolin-1-in-the-lung-of-bleomycin-induced-interstitial-lung-disease
#17
Yida Xing, Li Wang, Hongjiang Wang, Xiaodan Kong, Libin Zhan
BACKGROUND: Interstitial lung disease (ILD) is a disease with high mortality worldwide. Unfortunately, its prognosis is still very poor. Therefore, developing the target molecular is very important for ILD diagnosis and treatment. Caveolin-1 (Cav-1) can regulate the formation of fibrosis by linking to the signaling pathway of transforming growth factor-β1 (TGF-β1), which is generally considered to be the most effective approach to solve the problem of ILD. METHODS: The rat model of ILD was induced by disposable transtracheal injection of bleomycin hydrochloride...
August 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/28925574/radiographic-fibrosis-score-predicts-survival-in-systemic-sclerosis-associated-interstitial-lung-disease
#18
Reoto Takei, Machiko Arita, Shogo Kumagai, Yuhei Ito, Fumiaki Tokioka, Takashi Koyama, Rintaro Saito, Keisuke Nishimura, Hironobu Tokumasu, Tadashi Ishida
BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality. METHODS: We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area...
September 19, 2017: Respirology: Official Journal of the Asian Pacific Society of Respirology
https://www.readbyqxmd.com/read/28923086/usefulness-of-lung-ultrasound-b-lines-in-connective-tissue-disease-associated-interstitial-lung-disease-a-literature-review
#19
REVIEW
YuKai Wang, Luna Gargani, Tatiana Barskova, Dan E Furst, Marco Matucci Cerinic
Interstitial lung disease (ILD) is a major pulmonary manifestation of connective tissue disease (CTD), leading to significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) is presently considered the diagnostic gold standard for pulmonary fibrosis diagnosis and quantification in the clinical arena. However, not negligible doses of ionizing radiation limit the use of HRCT, especially for serial follow-up in younger female patients. In the past decade, lung ultrasound (LUS) has been proposed to assess ILD by detecting and quantifying sonographic B-lines...
September 18, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28918442/the-role-of-periostin-in-lung-fibrosis-and-airway-remodeling
#20
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
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