Abstract:
:Idiopathic pulmonary fibrosis (IPF), the most prevalent idiopathic interstitial pneumonia, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for optimal management. The recent ATS/ERS/JRS/ALAT recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway with formal designation of criteria for an HRCT pattern of UIP. In the correct clinical context, a UIP pattern on HRCT is indicative of a definite diagnosis of IPF without the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of guideline recommendations by clinicians has identified limitations that need to be addressed in future statements. Key problems include: 1) the lack of management recommendations for the highly prevalent clinical scenarios of probable and possible IPF; 2) the ongoing confusion about the diagnostic role of bronchoalveolar lavage (reflecting ambiguity in the current recommendation); 3) HRCT misdiagnosis by less experienced radiologists, increasingly recognised as a major problem; and 4) the lack of integration of clinical data, including the treated course of disease, in the designation of the diagnostic likelihood of IPF.
journal_name
Respir Resjournal_title
Respiratory researchauthors
Wells AUdoi
10.1186/1465-9921-14-S1-S2subject
Has Abstractpub_date
2013-01-01 00:00:00pages
S2eissn
1465-9921issn
1465-993Xpii
1465-9921-14-S1-S2journal_volume
14 Suppl 1pub_type
杂志文章abstract:BACKGROUND:The effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia (iNSIP), and factors affecting treatment outcome, are not fully understood. We aimed to investigate the long-term treatment response and factors affecting the treatment outcome in iNSIP patients from a ...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究
doi:10.1186/s12931-017-0686-7
更新日期:2017-12-06 00:00:00
abstract:BACKGROUND:Idiopathic pulmonary fibrosis (IPF) is a chronically progressive interstitial lung disease of unknown etiology. Previously, we have demonstrated the selective upregulation of the macrophage-derived chemokine CCL22 and the thymus activation-regulated chemokine CCL17 among chemokines, in a rat model of radiati...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-10-80
更新日期:2009-08-29 00:00:00
abstract::The interaction between viruses and dendritic cells (DCs) is varied and complex. DCs are key elements in the development of a host response to pathogens such as viruses, but viruses have developed survival tactics to either evade or diminish the immune system that functions to kill and eliminate these micro-organisms....
journal_title:Respiratory research
pub_type: 杂志文章,评审
doi:10.1186/rr63
更新日期:2001-01-01 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:Inspiratory activity is a prerequisite for successful application of patient triggered ventilation such as proportional assist ventilation (PAV). It has recently been reported that surfactant instillation increases the activity of slowly adapting pulmonary stretch receptors (PSRs) followed by a shorter inspi...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-7-38
更新日期:2006-03-10 00:00:00
abstract:BACKGROUND:To assess the symptomatic and cost burden among patients initiating long-acting bronchodilator (LABD) therapy and impact of adherence on healthcare resource use and costs. METHODS:This retrospective cohort study identified patients with COPD who were newly prescribed a LABD (long-acting muscarinic antagonis...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-015-0295-2
更新日期:2015-11-16 00:00:00
abstract::The transcription factors nuclear factor interleukin-6 (NF-IL6), early growth response-1 (EGR-1) and hypoxia-inducible factor-1 (HIF-1) have important roles in the molecular pathophysiology of hypoxia-associated pulmonary disease. NF-IL6 controls the production of interleukin (IL)-6 in vascular endothelial cells, whic...
journal_title:Respiratory research
pub_type: 杂志文章,评审
doi:10.1186/rr27
更新日期:2000-01-01 00:00:00
abstract:BACKGROUND:CpG oligodeoxynucleotides (CpG-ODN) are capable of inducing high amounts of type I IFNs with many immunomodulatory properties. Furthermore, type-I IFNs have been proposed to play a key role in mediating effects of CpG-ODN. The precise role of IFN-beta in the immunomodulatory effects of CpG-ODN is not known. ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-6-25
更新日期:2005-03-05 00:00:00
abstract:BACKGROUND:Debate continues as to whether acute bronchodilator responsiveness (BDR) predicts long-term outcomes in COPD. Furthermore, there is no consensus on a threshold for BDR. METHODS:At baseline and during the 4-year Understanding Potential Long-term Improvements in Function with Tiotropium (UPLIFT®) trial, patie...
journal_title:Respiratory research
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1186/1465-9921-12-6
更新日期:2011-01-11 00:00:00
abstract:BACKGROUND:Accumulation of profibrotic myofibroblasts in fibroblastic foci (FF) is a crucial process for development of fibrosis during idiopathic pulmonary fibrosis (IPF) pathogenesis, and transforming growth factor (TGF)-β plays a key regulatory role in myofibroblast differentiation. Reactive oxygen species (ROS) has...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-016-0420-x
更新日期:2016-08-30 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:In utero exposure to particulate matter with an aerodynamic diameter of less than 2.5 μm (PM2.5) has been linked to child lung function. Overlapping evidence suggests that child sex and exposure timing may modify effects and associations may be mediated through glutathione S-transferase P1 (GSTP1) methylatio...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-018-0774-3
更新日期:2018-04-27 00:00:00
abstract:BACKGROUND:Allergen-containing subpollen particles (SPP) are released from whole plant pollen upon contact with water or even high humidity. Because of their size SPP can preferentially reach the lower airways where they come into contact with surfactant protein (SP)-D. Our previous work demonstrated that SP-D increase...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-11-83
更新日期:2010-06-22 00:00:00
abstract:BACKGROUND:Bronchiolitis obliterans syndrome (BOS) after allogeneic hematopoietic stem cell transplantation (HSCT) is currently treated with systemic corticosteroids despite poor efficacy and side effects. This study investigated the therapeutic effect of budesonide/formoterol, montelukast and n-acetylcysteine, which a...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-016-0380-1
更新日期:2016-05-26 00:00:00
abstract:BACKGROUND:Nintedanib is an approved therapy for idiopathic pulmonary fibrosis (IPF). Some patients treated with nintedanib experience weight loss. Exploratory data suggest that low body mass index or weight loss are associated with worse outcomes in patients with IPF. We investigated whether BMI at baseline or weight ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-01528-4
更新日期:2020-11-25 00:00:00
abstract::The use of biomarkers in medicine lies in their ability to detect disease and support diagnostic and therapeutic decisions. New research and novel understanding of the molecular basis of the disease reveals an abundance of exciting new biomarkers who present a promise for use in the everyday clinical practice. The pas...
journal_title:Respiratory research
pub_type: 杂志文章,评审
doi:10.1186/1465-9921-6-78
更新日期:2005-07-21 00:00:00
abstract:BACKGROUND:Idiopathic pulmonary fibrosis (IPF) is a rare disease with a median survival of 3-5 years after diagnosis with limited treatment options. The aim of this study is to assess the psychometric characteristics of the Short Form 36 Health Status Questionnaire (SF-36) in IPF and to provide disease specific minimal...
journal_title:Respiratory research
pub_type: 临床试验,杂志文章
doi:10.1186/s12931-019-1010-5
更新日期:2019-03-01 00:00:00
abstract:RATIONALE:Information concerning how climate and atmospheric pollutants affects physical activity in COPD patients is lacking and might be valuable in determining when physical activity should be encouraged. METHODS:Seventy-three stable COPD patients recorded on daily diary cards worsening of respiratory symptoms, pea...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-015-0229-z
更新日期:2015-06-13 00:00:00
abstract:BACKGROUND:Current therapy for relieving bronchoconstriction may be ineffective in severe asthma, particularly in the small airways. The aim of this study was to further characterise responses to the recently identified novel bronchodilators rosiglitazone (RGZ) and chloroquine (CQ) under conditions where β-adrenoceptor...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-15-29
更新日期:2014-03-12 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:Acute respiratory distress syndrome (ARDS) is a severe form of lung injury characterized by damage to the epithelial barrier with subsequent pulmonary edema and hypoxic respiratory failure. ARDS is a significant medical problem in intensive care units with associated high care costs. There are many potential...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-018-0856-2
更新日期:2018-08-22 00:00:00
abstract:BACKGROUND:Alveolar macrophages are professional phagocytes that remove microbial pathogens inhaled into the lung. The phagocytic ability is compromised in chronic obstructive pulmonary disease (COPD). However, the molecular mechanisms underlying this defect in phagocytosis are not clearly defined. MATERIALS AND METHO...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-1297-2
更新日期:2020-01-28 00:00:00
abstract::The hygiene hypothesis states that childhood asthma develops as a result of decreased exposure to infectious agents during infancy and early childhood. This results in the persistence of the neonatal T helper lymphocyte 2 immunophenotype, thereby predisposing the child to atopic disease. While multiple studies support...
journal_title:Respiratory research
pub_type: 杂志文章,评审
doi:10.1186/rr81
更新日期:2001-01-01 00:00:00
abstract:BACKGROUND:There are limited series concerning Dieulafoy disease of the bronchus. We describe the clinical presentation of a series of 7 patients diagnosed with Dieulafoy disease of the bronchus and provide information about the pathological diagnosis approach. PATIENTS AND METHODS:A retrospective review of patients w...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-9-58
更新日期:2008-08-05 00:00:00
abstract:BACKGROUND:Obstructive sleep apnoea (OSA) and morbid obesity (MO), defined by a body mass index ≥35 kg/m2, are two closely related conditions. Recent studies suggest that circulating microRNA (miRNA) plays a potential role in the physiopathology of both conditions. To date, circulating miRNA expression has been studied...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-020-1302-9
更新日期:2020-02-04 00:00:00
abstract:BACKGROUND:Programming a mechanical ventilator with a biologically variable or fractal breathing pattern (an example of 1/f noise) improves gas exchange and respiratory mechanics. Here we show that fractal ventilation increases respiratory sinus arrhythmia (RSA) -- a mechanism known to improve ventilation/perfusion mat...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/1465-9921-6-41
更新日期:2005-05-09 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: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:Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with poor prognosis. The kinase inhibitor nintedanib specific for vascular endothelial growth factor receptor (VEGFR), platelet-derived growth factor receptor (PDGFR) and fibroblast growth factor receptor (FGFR) significantly reduced the rate ...
journal_title:Respiratory research
pub_type: 杂志文章
doi:10.1186/s12931-014-0157-3
更新日期:2014-12-12 00:00:00
abstract:BACKGROUND:Proteomic studies of respiratory disorders have the potential to identify protein biomarkers for diagnosis and disease monitoring. Utilisation of sensitive quantitative proteomic methods creates opportunities to determine individual patient proteomes. The aim of the current study was to determine if quantita...
journal_title:Respiratory research
pub_type: 杂志文章,随机对照试验
doi:10.1186/1465-9921-12-124
更新日期:2011-09-22 00:00:00