European Respiratory Journal最新文献

{"title":"Post Tuberculosis Lung Disease: questions and answers.","authors":"Giovanni Battista Migliori, Rosella Centis, Lia D'Ambrosio, Denise Rossato Silva, Daria Podlekareva, H Simon Schaaf","doi":"10.1183/13993003.00992-2025","DOIUrl":"https://doi.org/10.1183/13993003.00992-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559610","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Functional sequence variants of intergenic long non-coding RNA on Chromosome 17q21 are associated with asthma.","authors":"Kwei-Yan Liu, Jia-Jyun Sie, Yajing Gao, Yen-Li Lo, Chao-Chien Wu, Chin-Chou Wang, Chau-Chyun Sheu, Ruay-Sheng Lai, Sum-Yee Leung, Chi-Cheng Lin, Yu-Feng Wei, Ching-Hsiung Lin, Sheng-Hao Lin, Jeng-Yuan Hsu, Wei-Chang Huang, Chia-Cheng Tseng, Yung-Fa Lai, Meng-Hsuan Cheng, Huang-Chi Chen, Chih-Jen Yang, Yuan-Ting Hsu, Chian-Heng Su, Shih-Chang Hsu, Wen-Yu Chung, Ming-Tsuen Hsieh, Li-Chen Chen, Chih-Hsing Hung, Chon-Lin Lee, Ming-Shyan Huang, Yufeng Zhou, Cathy S J Fann, Shau-Ku Huang","doi":"10.1183/13993003.00847-2025","DOIUrl":"https://doi.org/10.1183/13993003.00847-2025","url":null,"abstract":"<p><strong>Background: </strong>The genetic and molecular basis of asthma remains unclear and its gene-environment interaction is still enigmatic. In the present study, we aimed to identify asthma-causing genetic variants and their interactions with the environment.</p><p><strong>Methods: </strong>We performed case-control genome-wide association studies on individuals of Han Chinese descent from the Taiwan biobank (case=4877 and control=(98 218) to identify asthma susceptibility loci, validated in a hospital-based population of subjects (N=2595). The 10- to 15-year exposure of cumulative ambient particulate matter with a diameter of less than 2.5 μm (PM_2.5) and polycyclic aromatic hydrocarbons (PAHs) were assessed for gene-environment relationships. The function of the newly identified long non-coding RNA, <i>lncZPBP2-3,</i> and its interaction with PM_2.5 and PAH exposure were analyzed using RNA immunoprecipitation, RNA pull-down, RT-qPCR, and western blotting.</p><p><strong>Findings: </strong>Chromosome 17q12-21 was found to be a significant risk region, encompassing variants of <i>lncZPBP2-3</i> and its neighboring genes, which interacted with increasing exposure to PM_2.5 and its adsorbed PAHs. The expression of <i>lncZPBP2-3</i> was elevated, correlating with the expression of its neighboring genes, in the peripheral blood of asthmatic individuals compared to that in controls. Unlike non-risk <i>lncZPBP2-3</i>, the risk variant of <i>lncZPBP2-3</i> disrupted the transcriptional suppression of the risk locus <i>via</i> its interaction with the transcription insulator, CCCTC-binding factor (CTCF), concomitant with the higher expression levels of neighboring genes in individuals with the risk genotype.</p><p><strong>Interpretation: </strong>An functional variant of lncRNA, lncZPBP2-3, was significantly associated with asthma and inducible by environmental PAH, suggesting a potentially novel genetic and molecular mechanism of asthma.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559593","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Interstitial Lung Abnormalities, Coronary Heart Disease, and Mortality.","authors":"Claire C Cutting, Jonathan A Rose, Ann-Marcia C Tukpah, Noriaki Wada, Mizuki Nishino, Sean Kalra, Matthew R Moll, Michael H Cho, Edwin K Silverman, Gregory L Kinney, Harry B Rossiter, Heida Bjarnadottir, Valborg Gudmundsdottir, Sigurdur Sigurdsson, Gunnar Gudmundsson, Vilmundur Gudnason, George R Washko, Matthew J Budoff, Hiroto Hatabu, Gary M Hunninghake, Rachel K Putman","doi":"10.1183/13993003.02286-2024","DOIUrl":"https://doi.org/10.1183/13993003.02286-2024","url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung abnormalities (ILA) share common risk factors with coronary heart disease (CHD), including increased age and cigarette smoking, however the relationship between ILA and CHD has not been well described.</p><p><strong>Methods: </strong>Participants from the Genetic Epidemiology of Chronic Obstructive Pulmonary Disease study (COPDGene) and Age Gene/Environment Susceptibility (AGES)-Reykjavik studies with ILA assessment, clinical CHD, and coronary artery calcium (CAC) data were included. In both cohorts, CHD was defined by clinical history and additionally by CAC>100. Multivariable logistic regression assessed the relationship between ILA and CHD; Cox proportional hazards models were used to assess mortality associated with ILA and CHD.</p><p><strong>Results: </strong>9% of subjects with CHD had ILA in both COPDGene and AGES-Reykjavik. Subjects with ILA had increased odds of CHD defined by clinical history in COPDGene (odds ratio [OR] 1.6, 95% confidence interval [CI]: 1.2 to 2.0, <i>p</i><0.001) and AGES-Reykjavik (OR 1.6, 1.2 to 2.0, <i>p</i><0.001), similar results were seen with CAC>100. In both COPDGene and AGES-Reykjavik, participants with CHD and ILA had a greater risk of death compared to those with CHD but without ILA (hazard ratio [HR] 2.0, 95% CI: 1.4 to 2.7, <i>p</i><0.001) and (HR 1.3, 95% CI: 1.1 to 1.4, <i>p</i><0.001), respectively. In AGES-Reykjavik, among participants with CHD, ILA was associated with an over 9-fold increase in the odds of a respiratory death (OR=9.6, 95% CI 3.2, 29, p<0.0001).</p><p><strong>Conclusion: </strong>ILA are a common co-occurrence with CHD and associated with worse mortality, suggesting that ILA are a clinically important comorbidity in patients with CHD.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Development and validation of a predictive 6-minute walk score in patients with Idiopathic Pulmonary Fibrosis.","authors":"Steven D Nathan, Jie Gao, Ho Cheol Kim, Abhimanyu Chandel, Henry Chen, Xiaomin Lu, Bernt van den Blink, Lixin Shao, Timothy R Watkins, Toby M Maher, Lisa Lancaster","doi":"10.1183/13993003.02565-2024","DOIUrl":"https://doi.org/10.1183/13993003.02565-2024","url":null,"abstract":"<p><strong>Background: </strong>The six minute walk test (6 MWT) provides an assessment of patient function and has been employed in interstitial lung disease clinical trials as an endpoint. The ISABELA studies were two replicate randomised controlled trials of IPF that included a regimented 6 MWT protocol. The goal of this study was to combine 6 MWT components into a pragmatic, easy to apply, composite clinical prediction score.</p><p><strong>Methods: </strong>6 MWT parameters associated with time to death or respiratory hospitalisation in the ISABELA studies were integrated into a single composite score. This score was then validated in an external cohort.</p><p><strong>Results: </strong>There were 1251 patients in the derivation set with 83 respiratory-related hospitalisations and 21 deaths observed after 48 weeks. After multivariable analysis, four parameters were independently predictive of outcomes: Borg dyspnoea score, oxygen flow rate, oxygen saturation nadir and the 6 MWT distance. A pragmatic model, termed the ODDS (oxygen, distance, dyspnoea, saturation) was then developed. This performed better than the individual parameters alone with an area under the curve (AUC) of 0.797, 0.781 and 0.766 for events at 12, 24, and 48 weeks, respectively. The ODDS model was similarly accurate when applied to the external validation set (N=295) at 48 weeks (AUC: 0.758; 95% CI: 0.688-0.825).</p><p><strong>Conclusion: </strong>The 6 MWT imparts important prognostic information which is best captured by combining constituent variables in a composite score system. The ODDS model might find utility in the clinical setting as well as in IPF studies where it can be used to risk stratify patients for outcomes.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559592","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Greater disease severity in adults with paediatric-onset <i>versus</i> adult-onset bronchiectasis: a multicenter EMBARC registry study.","authors":"Luai Khalaili, Stefano Aliberti, Kateryna Viligorska, Francesco Blasi, Nili Stein, Raya Cohen, Rafea Zoubi, Yochai Adir, Alessandro De Angelis, Benjamin Jaaming New, Lewis Marshall, James D Chalmers, M Shteinberg","doi":"10.1183/13993003.00665-2025","DOIUrl":"https://doi.org/10.1183/13993003.00665-2025","url":null,"abstract":"<p><strong>Background: </strong>Young adults with paediatric- onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterized. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).</p><p><strong>Methods: </strong>Data from four EMBARC (European Bronchiectasis Registry) centers were analyzed. POBE was defined as patient-reported symptom onset before 18 years, while AOBE was defined as symptom onset at or after 18 years. We compared POBE to AOBE and used multivariable models to identify factors associated with disease severity, including lung function, Pseudomonas aeruginosa infection, exacerbations, and hospitalizations.</p><p><strong>Results: </strong>Among 1422 patients, 249 (17.5%) had POBE (mean onset age: 6.5 years), and 1173 had AOBE (mean onset age: 55.4 years). POBE patients were younger at enrollment (50.3 <i>versus</i> 66 years), had longer disease duration (43.3 <i>versus</i> 10.8 years), worse lung function (FEV1% predicted: 70.8 <i>versus</i> 84.2), greater radiological extent (Reiff index: 6.0 <i>versus</i> 4.4), higher bacterial infection rates (72.3% <i>versus</i> 54.6%), and more exacerbations (median: 2 <i>versus</i> 1 per year) compared to AOBE (p<0.001 across all comparisons). Symptom duration was independently associated with <i>P. aeruginosa</i> infection, hospitalization, exacerbations, and reduced FEV1%. Congenital etiologies, such as primary ciliary dyskinesia and primary immune deficiencies, further contributed to disease severity.</p><p><strong>Conclusions: </strong>Adults with POBE exhibit greater disease severity than those with AOBE, likely due to prolonged symptom duration and congenital aetiologies. Conventional bronchiectasis severity scores may underestimate severity in young people with POBE. Optimized care, including structured transition to adult care, may mitigate progression in POBE patients.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-07-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559608","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"ERJ Podcast June 2025: Building translational bridges in idiopathic pulmonary fibrosis research.","authors":"","doi":"10.1183/13993003.e6506-2025","DOIUrl":"https://doi.org/10.1183/13993003.e6506-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"30 23 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144547674","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The relationship between a known diagnosis of tuberculosis and symptom reporting: implications for case detection strategies.","authors":"Alvin Kuo Jing Teo, Khanh Boi Luu, Frances Garden, Cuong Duc Pham, Jennifer Ho, Thu-Anh Nguyen, Guy B Marks, Greg J Fox","doi":"10.1183/13993003.02521-2024","DOIUrl":"https://doi.org/10.1183/13993003.02521-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144505291","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"European Respiratory Society and European Sleep Research Society Statement on the treatment of central sleep apnoea with adaptive servo-ventilation.","authors":"Winfried J Randerath, Sophia E Schiza, Michael Arzt, Maria Rosaria Bonsignore, Raphael Heinzer, Carolina Lombardi, Robert Lyssens, Gianfranco Parati, Thomas Penzel, Jean Louis Pepin, Dirk Pevernagie, Silke Ryan, Luca Vignatelli, Esther I Schwarz","doi":"10.1183/13993003.00263-2025","DOIUrl":"https://doi.org/10.1183/13993003.00263-2025","url":null,"abstract":"<p><p>Adaptive servo-ventilation (ASV) has been considered effective in controlling various forms of central sleep apnoea (CSA) and also any additional obstructive sleep apnoea (OSA) component. However, after the publication of the SERVE-HF study, its use was restricted in patients with systolic heart failure and prevalent CSA and was withheld from many patients with symptomatic CSA. In the meantime, the devices have been further developed and the algorithms adapted, and there is new evidence from randomised controlled trials and observational studies that makes it necessary to re-evaluate some societies' statements on the use of ASV, especially in patients with heart failure and CSA and with the current ASV devices. This short statement is based on a review of the effect of ASV on hard cardiovascular endpoints, echocardiographic parameters and exercise capacity as well as on sleep architecture and sleep quality, symptoms and quality of life (QoL) in patients with CHF. The expert group concludes that ASV has positive effects on CSA and quality of life in various forms of CSA that current ASV devices have no negative effect on hard cardiovascular endpoints and that ASV has positive effects on patient-reported outcomes. Moreover, it is used by TF members after optimal treatment of the underlying disease and after an unsuccessful CPAP trial in patients with heart failure with preserved ejection fraction, but also in patients with LVEF 30-45%. In the latter group, however, initiation is performed in expert centres only. In severe systolic heart failure, ASV is sometimes evaluated in a palliative therapy concept for severely symptomatic patients with CSA.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144505287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"European Respiratory Society Clinical Practice Guideline on Telemedicine in Home Mechanical Ventilation.","authors":"Marieke L Duiverman, Carla Ribeiro, Thomy Tonia, Anda Hazenberg, Stien van Meerloo, Hans van Meerloo, Stefanie Werther, Christopher Schöbel, Aylin Özsancak Uğurlu, Jean-Christian Borel, Christina Jácome, Maxime Patout, Karen Ward, Clare Williams, Begum Ergan, Chris Carlin, Patrick Murphy, Raphaela Dellacà, Michele Vitacca, Claudia Crimi","doi":"10.1183/13993003.00094-2025","DOIUrl":"https://doi.org/10.1183/13993003.00094-2025","url":null,"abstract":"<p><strong>Background: </strong>With the increasing prevalence of patients on home mechanical ventilation (HMV), changing indications, shortage of hospital resources, and rapidly evolving technology, there is an urgent need for evaluating the added value of telemedicine in initiation and follow-up of HMV. This European Respiratory Society (ERS) clinical practice guideline provides evidence-based recommendations on the use of telemedicine in HMV.</p><p><strong>Methods: </strong>The ERS Task Force consisted of 20 members, including a patient representative and her caregiver. The Task Force addressed five PICO questions and three narrative questions. Systematic searches were performed in MEDLINE, EMBASE, Cochrane, CINAHL. Evidence was synthesized by conducting meta-analyses, when possible, or when not, narratively. Certainty of evidence was rated with GRADE (Grading of Recommendations Assessment, Development and Evaluation). The \"evidence-to-decision\" framework was used to decide on the direction and formulate strengths of recommendations.</p><p><strong>Results: </strong>The panel makes a conditional recommendation for the initiation of HMV with telemedicine in patients with neuromuscular diseases and in patients with COPD. No recommendation could be made for obesity hypoventilation syndrome. The panel conditionally recommends the use of telemedicine for the follow-up of patients on HMV, although could not make recommendations on parameters to be monitored. Suggestions were mainly based on theoretical benefits and patient preferences, as our confidence in the evidence was low.</p><p><strong>Conclusions: </strong>With these guidelines, clinical practice recommendations are provided for the use of telemedicine in HMV. Technological advances and the use of advanced data processing algorithms and artificial intelligence were identified as drivers for future research and telemedicine use.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144505288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Genetic associations between Serotonin Receptor 1F (HTR1F) regulatory variation and sleep apnea in non-obese individuals: Insights from GWAS and eQTL analyses.","authors":"Satu Strausz,Martin Broberg,Samuel E Jones,Jukka Koskela,Tuomo Kiiskinen,FinnGen,,Aarno Palotie,Tuula Palotie,Adel Bachour,Richa Saxena,Samuli Ripatti,Erik Abner,Hanna M Ollila","doi":"10.1183/13993003.01778-2024","DOIUrl":"https://doi.org/10.1183/13993003.01778-2024","url":null,"abstract":"BACKGROUNDSleep apnea is a common sleep disorder affecting at least ten percent of the population. It is caused by lack of breathing during sleep, typically mediated by obstruction of airways or less frequently by misdirected central signals for breathing. The primary risk factor is a high body mass index (BMI), causing airway obstruction. However, understanding risk factors for sleep apnea in non-obese (BMI<30) individuals requires further exploration.AIMOur goal was to elucidate genetic risk factors for sleep apnea in non-obese individuals.METHODSWe performed genome-wide association testing in individuals with BMI<30 in FinnGen including 20 413 cases with sleep apnea diagnosis (ICD-10 G47.3 or ICD-9 3472) and 443 463 disease free controls. We replicated our analysis in Estonian Biobank.RESULTSWe identified a significant association within the Serotonin receptor 1F (HTR1F) locus (rs1818163, beta=0.059, se=0.010, p<1.58e-8), and replicated the association in Estonian Biobank (beta =0.042, se=0.020, p=0.034). The association signal co-localized with HTR1F expression across multiple tissues (posterior probability>0.8), and single cell sequencing implicated HTR1F expression particularly in neurons. Analysis of eQTL data further supported a possible regulatory role in neurons (beta=-0.03, p=1.2e-4). Finally, PheWAS analysis of rs1818163 showed an association with objectively measured sleep-activity data, specifically with the number of awakenings during the night (p=5.6e-8).CONCLUSIONSThe findings indicate association of HTR1F in sleep apnea particularly in the patient population within the non-obese BMI range and provide insight into the growing evidence of serotonin signaling as a factor modulating liability to sleep apnea.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"43 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-06-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144504617","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}