Abstract:
:A proportion of patients with certain types of interstitial lung disease (ILD), including chronic hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a progressive fibrosing phenotype that shows similarities in clinical course to idiopathic pulmonary fibrosis. Irrespective of the clinical diagnosis, these progressive fibrosing ILDs show commonalities in the underlying pathogenetic mechanisms that drive a self-sustaining process of pulmonary fibrosis. The natural history of progressive fibrosing ILDs is characterized by decline in lung function, worsening of symptoms and health-related quality of life, and early mortality. Greater impairment in forced vital capacity or diffusion capacity of the lungs for carbon monoxide, and a greater extent of fibrotic changes on a computed tomography scan, are predictors of mortality in patients with fibrosing ILDs. However, the course of these diseases is heterogenous and cannot accurately be predicted for an individual patient. Data from ongoing clinical trials and patient registries will provide a better understanding of the clinical course and impact of progressive fibrosing ILDs.
journal_name
Respir Resjournal_title
Respiratory researchauthors
Kolb M,Vašáková Mdoi
10.1186/s12931-019-1022-1subject
Has Abstractpub_date
2019-03-14 00:00:00pages
57issue
1eissn
1465-9921issn
1465-993Xpii
10.1186/s12931-019-1022-1journal_volume
20pub_type
杂志文章,评审abstract:BACKGROUND:Apoptosis has recently been proposed to contribute to the pathogenesis of emphysema. METHODS:In order to establish if cell fate plays a role even in end-stage disease we studied 16 lungs (9 smoking-associated and 7 alpha1antitrypsin (AAT)-deficiency emphysema) from patients who had undergone lung transplant...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-6-14
更新日期:2005-02-10 00:00:00
abstract:BACKGROUND:Exposure to ozone activates innate immune function and causes neutrophilic (PMN) airway inflammation that in some individuals is robustly elevated. The interplay between immuno-inflammatory function and genomic signaling in those with heightened inflammatory responsiveness to ozone is not well understood. O...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-13-89
更新日期:2012-10-03 00:00:00
abstract:BACKGROUND:The acute respiratory distress syndrome (ARDS) is characterized by the acute onset of hypoxemia and bilateral lung infiltrates in response to an inciting event, and is associated with high morbidity and mortality. Patients undergoing allogeneic hematopoietic stem cell transplantation (HSCT) are at increased ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-019-0981-6
更新日期:2019-01-21 00:00:00
abstract:BACKGROUND:Tracheal epithelial cells are anchored to a dynamic basement membrane that contains a variety of extracellular matrix proteins including collagens and laminins. During development, wound repair and disease of the airway epithelium, significant changes in extracellular matrix proteins may directly affect cell...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-7-105
更新日期:2006-08-02 00:00:00
abstract:BACKGROUND:Idiopathic pulmonary arterial hypertension (IPAH) continues to be one of the most serious intractable diseases that might start with activation of several triggers representing the genetic susceptibility of a patient. To elucidate what essentially contributes to the onset and progression of IPAH, we investig...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-13-103
更新日期:2012-11-17 00:00:00
abstract:RATIONALE:We had shown that COPD women expressed more dyspnea than men for the same degree of airway obstruction. OBJECTIVES:Evaluate gender differences in respiratory factors associated with dyspnea in COPD patients. METHODS:In a FEV1 % matched population of 100 men and women with COPD we measured: age, MMRC, FEV1, ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-8-18
更新日期:2007-03-06 00:00:00
abstract:BACKGROUND:Deep inspirations (DI) have bronchodilatory and bronchoprotective effects in healthy human subjects, but these effects appear to be absent in asthmatic lungs. We have characterized the effects of DI on lung mechanics during mechanical ventilation in healthy mice and in a murine model of acute and chronic air...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-9-23
更新日期:2008-02-28 00:00:00
abstract::Monitoring of pulmonary physiology is fundamental to the clinical management of patients with Cystic Fibrosis. The current standard clinical practise uses spirometry to assess lung function which delivers a clinically relevant functional readout of total lung function, however does not supply any visible or localised ...
journal_title:Respiratory research
pub_type: 杂志文章,评审
doi:10.1186/s12931-017-0578-x
更新日期:2017-05-17 00:00:00
abstract:BACKGROUND:Manual auscultation to detect abnormal breath sounds has poor inter-observer reliability. Digital stethoscopes with artificial intelligence (AI) could improve reliable detection of these sounds. We aimed to independently test the abilities of AI developed for this purpose. METHODS:One hundred and ninety two...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-01523-9
更新日期:2020-09-29 00:00:00
abstract::Respiratory diseases including asthma and chronic obstructive pulmonary disease (COPD) are characterised by excessive and persistent inflammation. Current treatments are often inadequate for symptom and disease control, and hence new therapies are warranted. Recent emerging research has implicated dyslipidaemia in pul...
journal_title:Respiratory research
pub_type: 杂志文章,评审
doi:10.1186/s12931-017-0526-9
更新日期:2017-02-28 00:00:00
abstract:BACKGROUND:Exacerbations of chronic obstructive pulmonary disease (COPD) are associated with loss of lung function and poor outcomes for patients. However, there are limited data on the time course of changes in forced expiratory volume in 1 s (FEV1) preceding the first reported symptom and after the start of an exacer...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1186/s12931-018-0944-3
更新日期:2018-12-13 00:00:00
abstract:BACKGROUND:Pleural effusion (PE) can be divided into benign pleural effusion (BPE) and malignant pleural effusion (MPE). There is no consensus on the identification of lung cancer-associated MPE using the optimal cut-off levels from five common tumor biomarkers (CEA, CYFRA 21-1, CA125, SCC-Ag, and NSE). Therefore, we a...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-01557-z
更新日期:2020-10-29 00:00:00
abstract:BACKGROUND:Systemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death. MAIN BODY:All patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including asse...
journal_title:Respiratory research
pub_type: 杂志文章,评审
doi:10.1186/s12931-019-0980-7
更新日期:2019-01-18 00:00:00
abstract:BACKGROUND:Preserved Ratio Impaired Spirometry (PRISm), defined as a reduced FEV1 in the setting of a preserved FEV1/FVC ratio, is highly prevalent and is associated with increased respiratory symptoms, systemic inflammation, and mortality. Studies investigating quantitative chest tomographic features, genetic associat...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-014-0089-y
更新日期:2014-08-06 00:00:00
abstract:BACKGROUND:Airway absorption and bioavailability of inhaled corticosteroids (ICSs) may be influenced by differences in pharmacokinetic properties such as lipophilicity and patient characteristics such as lung function. This study aimed to further investigate and clarify the distribution of budesonide and fluticasone in...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1186/1465-9921-10-104
更新日期:2009-10-31 00:00:00
abstract:BACKGROUND:It is generally accepted that emphysematous lungs are characterized by an increase in the numbers of neutrophils, macrophages, and CD8+ T lymphocytes, the lasts having increased cytotoxic activity. Because systemic inflammation is also a component of emphysema, we hypothesize that peripheral CD8+ T lymphocyt...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-8-62
更新日期:2007-09-06 00:00:00
abstract:BACKGROUND:Cysteine-containing leukotrienes (cysteinyl-LTs) are pivotal inflammatory mediators that play important roles in the pathophysiology of asthma, allergic rhinitis, and other inflammatory conditions. In particular, cysteinyl-LTs exert a variety of effects with relevance to the aetiology of asthma such as smoot...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-7-42
更新日期:2006-03-22 00:00:00
abstract:BACKGROUND:Macrolides reduce exacerbations in patients with COPD. Their effects on health status has not been assessed as primary outcome and is less clear. This study assessed the effects of prophylactic azithromycin on cough-specific health status in COPD-patients with chronic productive cough. METHODS:In this rando...
journal_title:Respiratory research
pub_type: 杂志文章,随机对照试验
doi:10.1186/1465-9921-14-125
更新日期:2013-11-14 00:00:00
abstract:BACKGROUND:Early life impairments leading to lower lung function by adulthood are considered as risk factors for chronic obstructive pulmonary disease (COPD). Recently, we compared the lung transcriptomic profile between two mouse strains with extreme total lung capacities to identify plausible pulmonary function deter...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-018-0795-y
更新日期:2018-06-06 00:00:00
abstract:BACKGROUND:Dual bronchodilator therapy is recommended for symptomatic patients with chronic obstructive pulmonary disease (COPD). There are limited data on effects of a combination of two long-acting bronchodilators on lung function including body plethysmography. METHODS:This multicentre, randomised, double-blind, si...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1186/s12931-016-0498-1
更新日期:2017-01-11 00:00:00
abstract:BACKGROUND:In asthma, higher chymase positive mast cell (MC-C) numbers are associated with less airway obstruction. In COPD, the distribution of MC-C and tryptase positive mast cells (MC-T) in central and peripheral airways, and their relation with lung function, is unknown. We compared MC-T and MC-C distributions in C...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-9-64
更新日期:2008-09-10 00:00:00
abstract:BACKGROUND:There is considerable heterogeneity in the rate of lung function decline in chronic obstructive pulmonary disease (COPD), the determinants of which are largely unknown. Observational studies in COPD indicate that low body mass index (BMI) is associated with worse outcomes, and overweight/obesity has a protec...
journal_title:Respiratory research
pub_type: 杂志文章,meta分析
doi:10.1186/s12931-019-1209-5
更新日期:2019-10-29 00:00:00
abstract:BACKGROUND:25-hydroxycholesterol (25-HC) is one of the oxysterols, which are oxidized derivatives of cholesterol, and has been reported to be involved in the pathogenesis of atherosclerosis and Alzheimer's disease. In lung, the possible involvement of 25-HC in airway diseases has been revealed. In the present study, we...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-13-63
更新日期:2012-07-31 00:00:00
abstract:BACKGROUND:Human beta-defensin-4 (hBD-4), a new member of the beta-defensin family, was discovered by an analysis of the genomic sequence. The objective of this study was to clarify hBD-4 expression in human lung tissue, along with the inducible expression in response to infectious stimuli, localization, and antimicrob...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-6-130
更新日期:2005-11-04 00:00:00
abstract:BACKGROUND:Skeletal muscle weakness in chronic obstructive pulmonary disease (COPD) is an important predictor of poor prognosis, but the molecular mechanisms of muscle weakness in COPD have not been fully elucidated. The aim of this study was to investigate the role of histone deacetylases(HDAC) in skeletal muscle weak...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-017-0588-8
更新日期:2017-05-19 00:00:00
abstract:BACKGROUND:Anxiety in patients with chronic obstructive pulmonary disease (COPD) is associated with self-reported disability. The purpose of this study is to determine whether there is an association between anxiety and functional measures, quality of life and dyspnea. METHODS:Data from 1828 patients with moderate to ...
journal_title:Respiratory research
pub_type: 杂志文章,随机对照试验
doi:10.1186/1465-9921-11-29
更新日期:2010-03-09 00:00:00
abstract:BACKGROUND:Retinoid X receptors (RXRs) are members of the nuclear receptor (NR) superfamily that mediate signalling by 9-cis retinoic acid, a vitamin A derivative. RXRs play key roles not only as homodimers but also as heterodimeric partners, e.g., for retinoic acid receptors, vitamin D receptors, and peroxisome prolif...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-018-0963-0
更新日期:2019-01-03 00:00:00
abstract:BACKGROUND:The prevalence and incidence of sarcoidosis varies worldwide. We estimated the prevalence and incidence of sarcoidosis in Korea using nationwide claims data from the Korean Health Insurance Review and Assessment Service. METHODS:Cases of sarcoidosis were identified for any visit between 2007 to 2016 that li...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-018-0871-3
更新日期:2018-08-28 00:00:00
abstract:BACKGROUND:The prevalence of pneumocystis pneumonia (PCP) and associated hypoxic respiratory failure is increasing in human immunodeficiency virus (HIV)-negative patients. However, no prior studies have evaluated the effect of early anti-PCP treatment on clinical outcomes in HIV-negative patient with severe PCP. Theref...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-019-1188-6
更新日期:2019-09-26 00:00:00
abstract:BACKGROUND:Secondary pulmonary alveolar septal formation requires platelet derived growth factor (PDGF-A) and platelet derived growth factor receptor-alpha (PDGFRα), and their regulation influences alveolar septal areal density and thickness. Insufficient PDGFRα expression in lung fibroblasts (LF) results in failed sep...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-14-68
更新日期:2013-07-02 00:00:00