Nicole Ng,Maria Molina-Molina,Ayodeji Adegunsoye,Raphael Borie,Chad A Newton,Benjamin Raby,David Zhang,Maria Padilla,Bruno Crestani,Marshall S Horwitz,Siobán Keel,Michael F Murray,Andrew B Stergachis,Shandra Knight,Christine K Garcia,Louise V Wain,Ganesh Raghu
{"title":"Genetics of Interstitial Lung Diseases: A State-of-the-Art Review.","authors":"Nicole Ng,Maria Molina-Molina,Ayodeji Adegunsoye,Raphael Borie,Chad A Newton,Benjamin Raby,David Zhang,Maria Padilla,Bruno Crestani,Marshall S Horwitz,Siobán Keel,Michael F Murray,Andrew B Stergachis,Shandra Knight,Christine K Garcia,Louise V Wain,Ganesh Raghu","doi":"10.1183/13993003.00788-2025","DOIUrl":"https://doi.org/10.1183/13993003.00788-2025","url":null,"abstract":"Advancements in genetics and genomics continue to further our understanding of their contributions to the development of interstitial lung diseases. This state-of-the-art clinical review synthesizes current knowledge of the contribution of genetics when evaluating patients suspected to have ILD. We consider highly penetrant Mendelian disorders as well as common variants conferring smaller risk that act in concert with other genetic and acquired risk factors. Additionally, gene-by-environment and pharmacogenomic interactions are discussed to highlight their impact on disease course. Lastly, the approach to genetic ILDs is reviewed from available testing to future directions.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"8 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144930249","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}
Isabella Annesi-Maesano, Hasan Bayram, Lorenzo Cecchi, Daniel Croft, Gennaro D'Amato, Arundhati Garud, Ozgecan Kayalar, Mehdi Misraedi, Subhabrata Moitra, Vanitha Sampath, Neeta Thakur, Kari Nadeau, John Balmes
{"title":"Natural disasters and respiratory health.","authors":"Isabella Annesi-Maesano, Hasan Bayram, Lorenzo Cecchi, Daniel Croft, Gennaro D'Amato, Arundhati Garud, Ozgecan Kayalar, Mehdi Misraedi, Subhabrata Moitra, Vanitha Sampath, Neeta Thakur, Kari Nadeau, John Balmes","doi":"10.1183/13993003.02563-2024","DOIUrl":"https://doi.org/10.1183/13993003.02563-2024","url":null,"abstract":"<p><p>Natural disasters-including heatwaves, wildfires, hurricanes, floods, earthquakes, and volcanic eruptions-significantly impact respiratory health, posing heightened risks to vulnerable populations such as individuals with pre-existing conditions, children, and the elderly. This review explores the complex relationship between natural catastrophes and respiratory health, emphasising the roles of chemical pollutants, biocontaminants, and meteorological factors.Epidemiological evidence highlights alarming trends, including increased asthma exacerbations, COPD hospitalisations, and respiratory infections following these events. During heatwaves, elevated ozone levels, and emissions from power generation for air conditioning exacerbate respiratory conditions, while fine and ultrafine particulate matter (PM), particularly during dust storms and wildfires, emerge as a major contributor to respiratory morbidity and mortality. Volcanic eruptions release hazardous gases, corrosive minerals, and plumes of particles and dust into the atmosphere, which exacerbate symptoms in individuals with pre-existing respiratory conditions. Thunderstorms often increase airborne pollen and mould concentrations, triggering episodes of thunderstorm asthma. Earthquake-damaged buildings are significant sources of dust, worsening respiratory symptoms among affected populations. Floods are the origin of mould proliferation, responsible for asthma and other respiratory diseases. First responders, such as firefighters, face acute and potentially chronic respiratory issues due to prolonged exposure to chemical pollutants and biocontaminants during rescue operations. Marginalised communities disproportionately bear the brunt of these health impacts due to systemic vulnerabilities and limited adaptive capacities.This review underscores the escalating respiratory health threats posed by natural disasters amid ongoing climate change. An integrated approach is needed to address these challenges through improved understanding, targeted interventions, and proactive measures to mitigate risks.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144948199","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}
Francesca Gonnelli,Heath Heatley,Ursie Smith,Neva Eleangovan,Vidya Navarantam,John Townend,Tamera J Corte,David B Price,Victoria Carter,Martina Bonifazi,Caitlin C Fermoyle,Richard Hubbard
{"title":"The impact of oral anticoagulants on idiopathic pulmonary fibrosis risk and prognosis: a population study.","authors":"Francesca Gonnelli,Heath Heatley,Ursie Smith,Neva Eleangovan,Vidya Navarantam,John Townend,Tamera J Corte,David B Price,Victoria Carter,Martina Bonifazi,Caitlin C Fermoyle,Richard Hubbard","doi":"10.1183/13993003.00432-2025","DOIUrl":"https://doi.org/10.1183/13993003.00432-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"17 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144930123","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":"CompEx Asthma: is it time for a change in clinical trials?","authors":"Tom M A Wilkinson,Praveen Akuthota","doi":"10.1183/13993003.00470-2025","DOIUrl":"https://doi.org/10.1183/13993003.00470-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"31 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144930122","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}
Miguel J Divo,Miguel A Martinez-Garcia,Monica Gonzalez,Francisco Campos-Rodriguez,Patricia Lloberes,Marta Marin-Oto,Marta Forner,David Sanz-Rubio,David Nieto,Bartolome R Celli,Jose M Marin,
{"title":"MACE or Death Risk in Obstructive Sleep Apnoea and the Effect of Positive Airway Pressure.","authors":"Miguel J Divo,Miguel A Martinez-Garcia,Monica Gonzalez,Francisco Campos-Rodriguez,Patricia Lloberes,Marta Marin-Oto,Marta Forner,David Sanz-Rubio,David Nieto,Bartolome R Celli,Jose M Marin, ","doi":"10.1183/13993003.00519-2025","DOIUrl":"https://doi.org/10.1183/13993003.00519-2025","url":null,"abstract":"RATIONALEEvidence regarding the efficacy of positive airway pressure (PAP) therapy in reducing the risks of non-fatal major cardiovascular events (NF-MACE) and mortality in patients with obstructive sleep apnoea (OSA) remains controversial.OBJECTIVESThis study aims to quantify the impact of PAP therapy on these risks and develop a predictive risk estimator.METHODSWe conducted a multicentre, observational, prospective study involving 5358 individuals diagnosed with OSA, with a median follow-up of 14 years (IQR 10-15 years). We derived and validated a risk estimator of NF-MACE (including myocardial infarction, stroke, revascularisation procedures, and congestive heart failure) and all-cause mortality, incorporating PAP adherence alongside clinical and sleep-related data.RESULTSThe cohort's mean (sd) for age was 55±11 years, the Body Mass Index 32.0±5.4 Kg·m-2, and an apnoea-hypopnea index (AHI) of 35 (±22) events/hour; 26% were females, and 1467 (37%) were PAP adherent. Over the follow-up period, 754 participants experienced NF-MACE, while 858 deaths were recorded. Significant predictors included prior cardiovascular events, non-HDL cholesterol ≥200 mg·dL-1, COPD diagnosis, AHI >30 events/hr, and age >60 years. PAP adherence was protective (OR 0.46; 95% CI: 0.38 to 0.56), and the absolute risk reduction varied depending on the baseline risk (median of 16%, IQR 12-18). The risk estimator yielded an AUROC of 0.75 and a Brier score of 0.17, with 64% sensitivity and 75% specificity.CONCLUSIONSPAP therapy is associated with long-term risk reduction of NF-MACE and mortality in OSA patients, while the developed risk estimator enhances clinical decision-making regarding therapy initiation.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"32 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144930125","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}
Reem Alluhibi,Aileen Marshall,David A Lomas,John R Hurst
{"title":"A Cure for Alpha-1? Novel Therapeutics in Alpha-1 Antitrypsin Deficiency.","authors":"Reem Alluhibi,Aileen Marshall,David A Lomas,John R Hurst","doi":"10.1183/13993003.01101-2025","DOIUrl":"https://doi.org/10.1183/13993003.01101-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"44 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144930124","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}
Hollian Richardson,Daniela Alferes De Lima Headley,Clare Clarke,Abirami Veluchamy,Petra Rauchhaus,Jennifer Pollock,Thomas Pembridge,Diane Cassidy,Holly R Keir,Simon Finch,Furrah Hussain,Margaret Band,Andrew Smith,Manish Patel,Mohammad Paracha,Gourab Choudhury,Devesh Dhasmana,Rekha Chaudhuri,Philip M Short,James D Chalmers
{"title":"The effect of different inhaled corticosteroid and long-acting bronchodilator combinations on the airway microbiome in patients with severe chronic obstructive pulmonary disease: A randomized trial (MUSIC).","authors":"Hollian Richardson,Daniela Alferes De Lima Headley,Clare Clarke,Abirami Veluchamy,Petra Rauchhaus,Jennifer Pollock,Thomas Pembridge,Diane Cassidy,Holly R Keir,Simon Finch,Furrah Hussain,Margaret Band,Andrew Smith,Manish Patel,Mohammad Paracha,Gourab Choudhury,Devesh Dhasmana,Rekha Chaudhuri,Philip M Short,James D Chalmers","doi":"10.1183/13993003.00287-2025","DOIUrl":"https://doi.org/10.1183/13993003.00287-2025","url":null,"abstract":"INTRODUCTIONThe microbiome is associated with exacerbation risk, quality of life and mortality in COPD. Inhaled corticosteroid (ICS) treatment has been reported to alter the microbiome through modulating host defence. How ICS alters the microbiome and whether effects are equal between different ICS preparations is debated. The aim of the MUSIC trial was to investigate whether commonly used ICS therapies have different effects on the airway microbiome in COPD.METHODSMulticentre randomized controlled trial. After a four-week washout period during which they withdrew from ICS, patients with COPD (FEV1 <50% predicted at baseline and/or a history of 2 or more exacerbations per year) were randomized to one of 4 treatments (budesonide/formoterol 400/12 mcg, fluticasone/salmeterol 500 mcg, fluticasone/salmeterol 250 mcg or aclidinium/formoterol 340/12 mcg, twice daily). Patients were followed-up for 3 months with monthly induced sputum, oropharyngeal and nasopharyngeal swabs for bacterial load and 16S rRNA sequencing to characterise the microbiome. Inflammatory markers were measured in sputum and blood. The primary outcome was bacterial load in oropharyngeal swabs comparing budesonide/formoterol versus fluticasone/salmeterol 500, with sputum bacterial load the key secondary endpoint.RESULTS122 participants started the washout period. ICS withdrawal was poorly tolerated, 61 participants withdrew before randomization with 45 experiencing an exacerbation. 61 patients were randomized. No statistically significant differences were observed for the primary comparison of budesonide/formoterol versus fluticasone/salmeterol 500 in oropharyngeal bacterial load. There was however a significant increase in sputum bacterial load with fluticasone/salmeterol 500 compared to budesonide/formoterol by month 3. This difference was not seen with fluticasone/salmeterol 250. No significant differences in microbiome alpha diversity were observed over time. Adverse events were similar between the groups.CONCLUSIONFluticasone/salmeterol 500 increased sputum but not upper airway bacterial loads. ICS withdrawal was poorly tolerated in severe COPD.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"32 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144930121","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}
Alvin Kuo Jing Teo, Khanh Boi Luu, Frances Garden, Cuong Duc Pham, Jennifer Ho, Thu-Anh Nguyen, Guy B Marks, Greg J Fox
{"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":"10.1183/13993003.02521-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12332464/pdf/","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":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Filters used for pulmonary function testing.","authors":"Roy T McKay","doi":"10.1183/13993003.00812-2025","DOIUrl":"https://doi.org/10.1183/13993003.00812-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"27 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144802574","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}
Barry J Plant, Gisli G Einarsson, Kevin F Deasy, Darren Dahly, Pradeep K Singh, Peter J Barry, Christopher H Goss, Isabelle Fajac, Tamara Vagg, Isabelle Durieu, Evelyn Flanagan, Grace O'Callaghan, Clémence Martin, Pierre-Régis Burgel, Charles S Haworth, R Andres Floto, Damian G Downey, Lieven J Dupont, Andrew M Jones, J Stuart Elborn, Joseph A Eustace, Marcus A Mall, Michael M Tunney
{"title":"Cystic Fibrosis Microbiome-directed Antibiotic Therapy Trial in Exacerbations Results Stratified (CFMATTERS): results of a multicentre randomised controlled trial.","authors":"Barry J Plant, Gisli G Einarsson, Kevin F Deasy, Darren Dahly, Pradeep K Singh, Peter J Barry, Christopher H Goss, Isabelle Fajac, Tamara Vagg, Isabelle Durieu, Evelyn Flanagan, Grace O'Callaghan, Clémence Martin, Pierre-Régis Burgel, Charles S Haworth, R Andres Floto, Damian G Downey, Lieven J Dupont, Andrew M Jones, J Stuart Elborn, Joseph A Eustace, Marcus A Mall, Michael M Tunney","doi":"10.1183/13993003.02443-2024","DOIUrl":"10.1183/13993003.02443-2024","url":null,"abstract":"<p><strong>Background: </strong>This study explores the effectiveness and safety of microbiome-directed antimicrobial therapy <i>versus</i> usual antimicrobial therapy in adult cystic fibrosis pulmonary exacerbations.</p><p><strong>Methods: </strong>A multicentre two-arm parallel randomised control trial conducted across Europe/North-America enrolled 223 participants (January 2015 to August 2017). All participants were chronically colonised with <i>Pseudomonas aeruginosa</i> and were randomised 1:1 into two study arms. The \"usual therapy\" group received 2 weeks of intravenous ceftazidime 3 g thrice daily (for allergies: aztreonam 2 g thrice daily) and tobramycin 5-10 mg·kg<sup>-1</sup> once daily. The \"microbiome-directed\" group received the same usual therapy plus an additional antibiotic with greatest presumed activity against the second, third and fourth most abundant genera present in the sputum microbiome, selected by a consensus expert treatment panel. The primary outcome was change in percentage of predicted forced expiratory volume in 1 s (ppFEV<sub>1</sub>) at 14 days post initiation of antibiotics. Secondary outcomes examined ppFEV<sub>1</sub> at 7 days, 28 days and 3 months; time to next exacerbation; symptom burden at 7 days; health-related quality of life (HRQoL) at 28 days; and number of exacerbations and <i>i.v.</i> antibiotic days at 12 months.</p><p><strong>Results: </strong>149 participants had an eligible exacerbation (usual therapy n=83, microbiome-directed therapy n=66). There was no difference between the groups for ppFEV<sub>1</sub> at day 14 (-1.1%, 95% CI -3.9-1.7%; p=0.46), or ppFEV<sub>1</sub> measured at other time points, or for time to next exacerbation (microbiome-directed <i>versus</i> usual therapy hazard ratio 0.91, 95% CI 0.60-1.38; p=0.66). The microbiome-directed group trended to have more <i>i.v.</i> days (median 42 days <i>versus</i> 28 days; p=0.08) and more subsequent exacerbations (median three <i>versus</i> two; p=0.044) the following year. There were no appreciable differences in symptom burden; however, HRQoL subscores were consistently worse in the microbiome-directed group (-4.3 points <i>versus</i> usual therapy, 95% CI -8.3--0.3 points; p=0.033).</p><p><strong>Conclusion: </strong>The addition of a third antibiotic based on sputum microbiome sequencing analysis did not result in improved clinical outcomes.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12332466/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144283157","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}