Abstract:
RESEARCH QUESTION:There is no widely accepted grading system for IPF disease severity, although physiologic impairment based on pulmonary function testing is frequently employed. We sought to describe clinical and functional characteristics as well as outcomes of patients with severe physiologic impairment. PATIENTS AND METHODS:IPF patients with severe physiologic impairment defined by FVC ≤ 50% and/or DLco ≤ 30% predicted evaluated in the Inova Advanced Lung Disease Program between 2011 and 2019 were included. Demographic, physiologic, functional treatment and outcome data were collated. RESULTS:There were 531 patients with IPF evaluated of whom 242 (46%) had severe physiologic impairment. Mean age was 72 ± 8 years; baseline FVC was 53 ± 17% and DLCO 28 ± 9% of predicted. The mean 6 min walks test (6MWT) distance was 304 ± 121 m with 59% of the patients requiring supplemental oxygen ([Formula: see text] group). There was a poor correlation between the 6MWT distance and both FVC% and DLco%. Patients in the 6MWTRA group had a better transplant-free survival than the [Formula: see text] group (p = 0.002). Patients managed before October 2014 and not receiving antifibrotic therapy had worse outcomes with reduced transplant-free survival compared with patients presenting after this date who did receive antifibrotic therapy (n = 113) (log rank p < 0.0001). CONCLUSION:IPF patients often present with severe physiologic impairment which may be poorly correlated with their functional status. Assessment of IPF disease severity should not be based on physiologic impairment alone, but should also encompass functional status as well as need for supplemental oxygen. Antifibrotic therapy in patients with severe physiologic impairment is associated with improved outcomes.
journal_name
Respir Resjournal_title
Respiratory researchauthors
Pastre J,Barnett S,Ksovreli I,Taylor J,Brown AW,Shlobin OA,Ahmad K,Khangoora V,Aryal S,King CS,Nathan SDdoi
10.1186/s12931-020-01600-zsubject
Has Abstractpub_date
2021-01-06 00:00:00pages
5issue
1eissn
1465-9921issn
1465-993Xpii
10.1186/s12931-020-01600-zjournal_volume
22pub_type
杂志文章abstract:BACKGROUND:The coexistence of COPD and asthma is widely recognized but has not been well described. This study characterizes clinical features, spirometry, and chest CT scans of smoking subjects with both COPD and asthma. METHODS:We performed a cross-sectional study comparing subjects with COPD and asthma to subjects ...
journal_title:Respiratory research
pub_type: 临床试验,杂志文章,多中心研究
doi:10.1186/1465-9921-12-127
更新日期:2011-09-27 00:00:00
abstract::The role of autoimmunity targeting epithelial antigens in asthma has been suggested, in particular in non-atopic and severe asthma. Periplakin, a desmosomal component, is involved in epithelial cohesion and intracellular signaling. We detected anti-periplakin IgG antibodies in 47/260 (18 %) patients with asthma, with ...
journal_title:Respiratory research
pub_type: 信件
doi:10.1186/s12931-016-0441-5
更新日期:2016-10-07 00:00:00
abstract:BACKGROUND:MIF is a critical mediator of the host defense, and is involved in both acute and chronic responses in the lung. Neutralization of MIF reduces neutrophil accumulation into the lung in animal models. We hypothesized that MIF, in the alveolar space, promotes neutrophil accumulation via activation of the CD74 r...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-10-33
更新日期:2009-05-04 00:00:00
abstract:BACKGROUND:The relationship between the Epworth sleepiness scale (ESS) and the apnea-hypopnea index (AHI) is uncertain and even poor. The major problem associated with the ESS might be a lack of consideration of weight in prediction in clinical practice. Would awarding different item-scores to the four scales of ESS it...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-01417-w
更新日期:2020-06-12 00:00:00
abstract:BACKGROUND:Hypoxia-induced mitogenic factor (HIMF) is developmentally regulated and plays an important role in lung pathogenesis. We initially found that HIMF promotes vascular tubule formation in a matrigel plug model. In this study, we investigated the mechanisms which HIMF enhances expression of vascular endothelial...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-7-37
更新日期:2006-03-02 00:00:00
abstract:BACKGROUND:Tobacco smoke and genetic susceptibility are risk factors for asthma and wheezing. The aim of this study was to investigate whether there is a combined effect of interleukin-13 gene (IL13) polymorphisms and tobacco smoke on persistent childhood wheezing and asthma. METHODS:In the Isle of Wight birth cohort ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-9-2
更新日期:2008-01-10 00:00:00
abstract:BACKGROUND:Chitosan, a polymer derived from chitin, has been used for nasal drug delivery because of its biocompatibility, biodegradability and bioadhesiveness. Theophylline is a drug that reduces the inflammatory effects of allergic asthma but is difficult to administer at an appropriate dosage without causing adverse...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-7-112
更新日期:2006-08-24 00:00:00
abstract:RATIONALE:Smoking-induced chronic obstructive pulmonary disease (COPD) is associated with acquired systemic cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction. Recently, sweat evaporimetry has been shown to efficiently measure β-adrenergic sweat rate and specifically quantify CFTR function in the se...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-15-25
更新日期:2014-02-25 00:00:00
abstract:BACKGROUND:WNT/β-catenin signaling plays an important role in the pathogenesis of idiopathic pulmonary fibrosis (IPF); however, the role of WNT10A via transforming growth factor (TGF)-β signaling remains unclear. METHODS:We evaluated the expression of WNT10A and TGF-β in bleomycin (BLM)-treated mice and the interactio...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-016-0357-0
更新日期:2016-04-12 00:00:00
abstract:BACKGROUND:Little is known about the association between cigarette smoking and asthma severity. We assessed smoking as a determinant of disease severity and control in a cohort of clinic-referred allergic subjects who developed new onset asthma. METHODS:Allergic rhinitis subjects with no asthma (n = 371) were followed...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-12-16
更新日期:2011-01-24 00:00:00
abstract:BACKGROUND:Chronic obstructive pulmonary disease (COPD) is a progressive and irreversible chronic inflammatory disease of the lung. The nature of the immune reaction in COPD raises the possibility that IL-17 and related cytokines may contribute to this disorder. This study analyzed the expression of IL-17A and IL-17F a...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-12-43
更新日期:2011-04-10 00:00:00
abstract:BACKGROUND:Previously we showed that reduced availability of the essential amino acid tryptophan per se attenuates post-transcriptional control of interleukin (IL)-6 and IL-8 leading to hyperresponsive production of these inflammatory mediators by airway epithelial cells. Availability of the non-essential amino acid ar...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-10-62
更新日期:2009-07-03 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:It has been confirmed that NF-κB p65 signaling pathway is involved in the regulation of alveolar hypercoagulation and fibrinolysis inhibition in acute respiratory distress syndrome (ARDS). Whether SN50, a NF-κB cell permeable inhibitor, could attenuate alveolar hypercoagulation and fibrinolysis inhibition in...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-01372-6
更新日期:2020-05-27 00:00:00
abstract:BACKGROUND:The home is the predominant environment for exposure to many environmental irritants such as air pollutants and allergens. Exposure to common indoor irritants including volatile organic compounds, formaldehyde and nitrogen dioxide, may increase the risk of snoring for children. The aim of this study was to i...
journal_title:Respiratory research
pub_type: 杂志文章,随机对照试验
doi:10.1186/1465-9921-5-19
更新日期:2004-11-04 00:00:00
abstract:BACKGROUND:The variability in the inflammatory burden of the lung in cystic fibrosis (CF) patients together with the variable effect of glucocorticoid treatment led us to hypothesize that glucocorticoid receptor (GR) gene polymorphisms may affect glucocorticoid sensitivity in CF and, consequently, may contribute to var...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究
doi:10.1186/1465-9921-8-88
更新日期:2007-11-29 00:00:00
abstract:BACKGROUND:During a lipopolysaccharide-induced lung inflammation, a massive accumulation of neutrophils occurs, which is normally cleared by macrophage phagocytosis following neutrophil apoptosis. However, in cases of extensive apoptosis the normal clearance system may fail, resulting in extensive neutrophil secondary ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-7-143
更新日期:2006-12-14 00:00:00
abstract:BACKGROUND:The long-term efficacy and safety of aclidinium bromide, a novel, long-acting muscarinic antagonist, were investigated in patients with moderate to severe chronic obstructive pulmonary disease (COPD). METHODS:In two double-blind, 52-week studies, ACCLAIM/COPD I (n=843) and II (n=804), patients were randomis...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1186/1465-9921-12-55
更新日期:2011-04-26 00:00:00
abstract:BACKGROUND:Pulmonary arterial hypertension (PAH) is associated with inflammatory response but it is unknown whether it is associated with alterations in NNMT activity and MNA plasma concentration. Here we examined changes in NNMT-MNA pathway in PAH in rats and humans. METHODS:PAH in rats was induced by a single subcut...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-016-0423-7
更新日期:2016-08-31 00:00:00
abstract:BACKGROUND:Symptoms of chronic obstructive pulmonary disease may vary throughout the day and it is important that therapeutic approaches provide 24-h symptom control. We report the results of two phase IIIb crossover studies, PT003011 and PT003012, investigating the 24-h lung function profile of GFF MDI (glycopyrrolate...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1186/s12931-017-0636-4
更新日期:2017-08-18 00:00:00
abstract:BACKGROUND:Induced and spontaneous sputum are used to evaluate the airways microbiota. Whether the sputum types can be used interchangeably in microbiota research is unknown. Our aim was to compare microbiota in induced and spontaneous sputum from COPD patients sampled during the same consultation. METHODS:COPD patien...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-017-0645-3
更新日期:2017-08-29 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:Azithromycin (Azm) is a macrolide recognized for its disease-modifying effects and reduction in exacerbation of chronic airway diseases. It is not clear whether the beneficial effects of Azm are due to its anti-microbial activity or other pharmacological actions. We have shown that Azm affects the integrity ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-019-1101-3
更新日期:2019-06-24 00:00:00
abstract::The attraction of leukocytes from circulation to inflamed lungs depends on the activation of both the leukocytes and the resident cells within the lung. In this study we determined gene expression and secretion patterns for monocyte chemoattractant protein-1 (MCP-1/CCL2) and T-cell specific CXCR3 agonistic chemokines ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-6-75
更新日期:2005-07-20 00:00:00
abstract:BACKGROUND:Exposure to crystalline silica is considered to increase the risk of lung fibrosis. The primary effector cell, the myofibroblast, plays an important role in the deposition of extracellular matrix (ECM). DNA methylation change is considered to have a potential effect on myofibroblast differentiation. Therefor...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-017-0576-z
更新日期:2017-05-12 00:00:00
abstract:BACKGROUND:Recent consensus statements demonstrate the breadth of the chronic rhinosinusitis (CRS) differential diagnosis. However, the classification and mechanisms of different CRS phenotypes remains problematic. METHOD:Statistical patterns of subjective and objective findings were assessed by retrospective chart re...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-6-149
更新日期:2005-12-19 00:00:00
abstract:BACKGROUND:Pulmonary sarcoidosis is an inflammatory disease, characterized by an accumulation of CD4+ lymphocytes and the formation of non-caseating epithelioid cell granulomas in the lungs. The disease either resolves spontaneously or develops into a chronic disease with fibrosis. The neurotrophins nerve growth factor...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-11-156
更新日期:2010-11-08 00:00:00
abstract:BACKGROUND:An increasing number of epidemiological and experimental studies have associated exposure to second-hand smoke (SHS) during pregnancy with adverse outcomes in newborns. As we have previously shown in mice, in utero exposure to SHS at critical stages of fetal development, results in altered lung responses and...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-017-0591-0
更新日期:2017-06-27 00:00:00
abstract:BACKGROUND:Steroid resistant (SR) asthma is characterized by persistent airway inflammation that fails to resolve despite treatment with high doses of corticosteroids. Furthermore, SR patient airways show increased numbers neutrophils, which are less responsive to glucocorticoid. The present study seeks to determine wh...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-016-0462-0
更新日期:2016-11-16 00:00:00
abstract:BACKGROUND:Chronic respiratory diseases (CRDs) are leading causes of morbidity worldwide. However, the spatial and temporal trends in prevalence and incidence of CRDs have not been estimated. METHODS:Based on data from the Global Burden of Diseases, Injuries, and Risk Factors Study 2017, we analyzed the prevalence and...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-1291-8
更新日期:2020-02-11 00:00:00