ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.01674-2025
Lidia Perea, Julieta Viglino, Belén Solarat, Pau Marrades, Sandra Casas-Recasens, Núria Mendoza, Pilar Martínez-Olondris, Rosanel Amaro, Alvar Agustí, Amelia Shoemark, Stefano Aliberti, James D Chalmers, Oriol Sibila, Rosa Faner
{"title":"Blood DNA methylation profile is altered in severe bronchiectasis.","authors":"Lidia Perea, Julieta Viglino, Belén Solarat, Pau Marrades, Sandra Casas-Recasens, Núria Mendoza, Pilar Martínez-Olondris, Rosanel Amaro, Alvar Agustí, Amelia Shoemark, Stefano Aliberti, James D Chalmers, Oriol Sibila, Rosa Faner","doi":"10.1183/23120541.01674-2025","DOIUrl":"10.1183/23120541.01674-2025","url":null,"abstract":"<p><strong>Rationale: </strong>Bronchiectasis is a chronic lung respiratory disease whose systemic aspect is poorly understood. We aimed to characterise the blood DNA methylation profiles of bronchiectasis patients and to determine the relationship of these profiles with disease severity and their functional relevance to circulating immune cells.</p><p><strong>Methods: </strong>Whole-genome DNA methylation was measured in blood from 165 bronchiectasis patients (Illumina 850k). The cohort was divided into discovery (n=94) and validation (n=71) sets. Differentially methylated positions (DMPs) associated with the Bronchiectasis Severity Index (BSI) were determined using sex-adjusted linear models. To explore functional relevance, whole-blood RNA sequencing was performed in a subset of patients. Blood immune cell proportions deconvoluted from methylation data were identified, clustered with gene expression and linked to BSI variables. An elastic net model was constructed to validate the results.</p><p><strong>Results: </strong>We identified 100 BSI-associated DMPs, 98% of which were hypomethylated in severe patients (BSI score ≥9). BSI-associated DMPs were enriched in leukocyte activation, secretion, immunoglobulin production and tissue remodelling. Of these DMPs, 15 (annotated to 16 genes) exhibited significant correlations between methylation and gene expression levels. Three of them (<i>PLXNC1</i>, <i>IL4R</i> and <i>MAP3K20</i>) were associated with neutrophils and BSI. Deconvoluted blood neutrophil proportions were correlated with chronic <i>Pseudomonas aeruginosa</i> infection, hospitalisations and dyspnoea, and negatively with forced expiratory volume in 1 s and female sex. The elastic net validated the BSI-associated DMPs in the second dataset.</p><p><strong>Conclusion: </strong>Severe bronchiectasis patients presented an altered blood epigenetic profile, which was influenced by blood neutrophils. These findings suggest that bronchiectasis has systemic implications, especially for the most severely affected patients.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617582/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073851","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.01671-2025
Dinah Hertig, Michael Furian, Mona Lichtblau, Julian Müller, Simon R Schneider, Stéphanie Saxer, Joel Herzig, Meret Bauer, Mirjam Schmucki, Diego Saragoni, Esther I Schwarz, Elizabeth Cajamarca, Rodrigo Hoyos, Silvia Ulrich
{"title":"Effect of supplemental oxygen therapy on 6-min walk distance in patients with pulmonary arterial hypertension living at 2850 m: a randomised, crossover trial.","authors":"Dinah Hertig, Michael Furian, Mona Lichtblau, Julian Müller, Simon R Schneider, Stéphanie Saxer, Joel Herzig, Meret Bauer, Mirjam Schmucki, Diego Saragoni, Esther I Schwarz, Elizabeth Cajamarca, Rodrigo Hoyos, Silvia Ulrich","doi":"10.1183/23120541.01671-2025","DOIUrl":"10.1183/23120541.01671-2025","url":null,"abstract":"<p><strong>Background: </strong>Patients with pulmonary arterial hypertension (PAH) who permanently live at high altitude (>2500 m) face particular challenges due to hypobaric hypoxia, which may exacerbate pulmonary hypertension and impair exercise capacity. We therefore investigated the effect of low-dose supplemental oxygen therapy (SOT) on 6-min walk distance (6MWD) in high-altitude residents diagnosed with PAH.</p><p><strong>Methods: </strong>Adults diagnosed with PAH who permanently live at >2500 m performed two 6MWD tests, once with SOT (1-2 L·min<sup>-1</sup> <i>via</i> nasal cannula provided by a portable back-packed concentrator) and once while breathing ambient air (air) at 2850 m according to a randomised, controlled, crossover trial. The primary outcome was the difference in 6MWD between SOT and air. Secondary outcomes were effects of SOT on heart rate, blood pressure and peripheral oxygen saturation (<i>S</i> <sub>pO<sub>2</sub></sub> ) and Borg scale.</p><p><strong>Results: </strong>28 patients (86% women, mean±sd age 45±12 years, resting <i>S</i> <sub>pO<sub>2</sub></sub> 93±3%, mean pulmonary artery pressure 52±16 mmHg, 6MWD at 2850 m 462±100 m) were included. SOT compared to hypoxic air at 2850 m did not improve 6MWD (mean difference -5 m, 95% CI: -24 to 14 m, p=0.576), dyspnoea, leg discomfort or end-exercise <i>S</i> <sub>pO<sub>2</sub></sub> , but did increase resting <i>S</i> <sub>pO<sub>2</sub></sub> .</p><p><strong>Conclusion: </strong>In high-altitude residents diagnosed with PAH, low-dose SOT as commonly prescribed <i>via</i> portable concentrator did not improve 6MWD nor dyspnoea perception, most likely due to insufficient flow provided by the concentrator during exercise, further lowered by exertional mouth breathing and the weight of the backpacked concentrator.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617584/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073848","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.00510-2026
Matthew Liu, Ryan C Murphy, Ashley Mazzotta, Ying Lai, Jonathan Lee, Stephen R Reeves, Teal S Hallstrand
{"title":"Relationships between basement membrane zone thickness and airway physiology, epithelial gene expression and innate immune cell infiltration in asthma.","authors":"Matthew Liu, Ryan C Murphy, Ashley Mazzotta, Ying Lai, Jonathan Lee, Stephen R Reeves, Teal S Hallstrand","doi":"10.1183/23120541.00510-2026","DOIUrl":"10.1183/23120541.00510-2026","url":null,"abstract":"<p><strong>Rationale: </strong>Thickening of the basement membrane zone (BMZ) has often been identified in airway biopsies of individuals with asthma, but the relationship of BMZ thickness to airway dysfunction and inflammation is incompletely understood. The objectives of the present study were to characterise the relationship between BMZ thickness and airway physiology, immune cell infiltration and airway gene expression.</p><p><strong>Methods: </strong>We used design-based stereology to measure BMZ thickness in endobronchial biopsy samples obtained from 10 healthy control subjects and 31 individuals with asthma not receiving asthma-directed anti-inflammatory therapies (only using as-needed albuterol for the study period). BMZ thickness was correlated with measures of airway physiology, sputum inflammatory markers, airway wall immunopathology and gene expression from airway epithelial brushings.</p><p><strong>Results: </strong>Individuals with asthma had a significantly thicker BMZ relative to healthy controls. BMZ thickness was negatively correlated with age and positively correlated with higher baseline lung function (forced vital capacity), mast cell (MC) infiltration of the airway epithelial compartment and expression of type 2 (T2) inflammatory genes in the airway. Thickening of the BMZ was not significantly associated with the bronchodilator response, direct airway hyperresponsiveness (AHR) to methacholine, indirect AHR in the form of exercise-induced bronchoconstriction, T1 or T3 inflammation, or airway eosinophilia.</p><p><strong>Conclusions: </strong>MC infiltration of the airway epithelial compartment and T2 inflammation are significantly associated with a thickened BMZ in asthma.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617596/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073820","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.01694-2025
Panayiotis Kouis, Mathieu Bottier, Nisreen Rumman, Mia Shanks, Joren Buekers, Dimitrios Megaritis, Andreas Protopapas, Amelia Shoemark, Myrofora Goutaki, Claire Jackson
{"title":"Systematic review of artificial intelligence and digital-automated tools for screening, diagnosis and characterisation of primary ciliary dyskinesia.","authors":"Panayiotis Kouis, Mathieu Bottier, Nisreen Rumman, Mia Shanks, Joren Buekers, Dimitrios Megaritis, Andreas Protopapas, Amelia Shoemark, Myrofora Goutaki, Claire Jackson","doi":"10.1183/23120541.01694-2025","DOIUrl":"10.1183/23120541.01694-2025","url":null,"abstract":"<p><strong>Background: </strong>Primary ciliary dyskinesia (PCD) is a rare, multigenic disorder of impaired mucociliary clearance leading to a spectrum of disease including chronic respiratory infection and bronchiectasis. PCD is phenotypically variable causing diagnostic challenges. PCD is underdiagnosed, and when it is made, diagnosis is often delayed. We describe evidence for use of digital-automated applications and/or artificial intelligence (AI) to improve PCD screening, diagnosis and characterisation.</p><p><strong>Methods: </strong>A systematic literature search (2004-2025) was conducted across PubMed, Embase and BioRxiv/MedRxiv (PROSPERO:CRD42024605689) by members of the ERS BEAT-PCD Clinical Research Collaboration (CRC). Screening and data extraction was performed by two independent reviewers and findings summarised qualitatively. In collaboration with the digital health ERS CONNECT CRC network, a narrative discussion focused on steps to real-world implementation.</p><p><strong>Results: </strong>Of 770 screened abstracts, 73 full-texts were assessed and 28 PCD-relevant studies with automated digital and/or AI-driven tools were included in the qualitative synthesis. Tools for enhanced ciliary function, ciliary ultrastructure and ciliary protein immunofluorescence analysis were presented in 17 studies. 11 papers presented tools to screen e-health records for PCD, stratify patients by genotype and phenotype, or characterise computed tomography parameters.</p><p><strong>Conclusions: </strong>Tools are available that could improve PCD diagnostic accuracy and reduce time-to-diagnosis. Studies were often single-centre, retrospective and of small sample size. The development of automated-digital tools or AI-driven tools requires representative patient datasets, human expert judgement to interpret and continual safety auditing. External validation is paramount before adaptation of healthcare systems, with adherence to EU AI Act framework to ensure accountability and safeguard against misuse. Simplified tools and philanthropic partnerships could facilitate implementation of new systems in resource limited settings.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617595/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073779","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.01606-2025
Etienne-Marie Jutant, Benoit Delarche, Magali Croquette, Antoine Beurnier, Alexandre Diaz, Vanessa Bironneau, Fabrice Caron, Marianne Riou, Athénaïs Boucly, Xavier Jaïs, Laurent Savale, Olivier Sitbon, Laurent Bertoletti, Marc Humbert, David Montani
{"title":"Pulmonary vascular reactivity in severe pulmonary hypertension associated with COPD.","authors":"Etienne-Marie Jutant, Benoit Delarche, Magali Croquette, Antoine Beurnier, Alexandre Diaz, Vanessa Bironneau, Fabrice Caron, Marianne Riou, Athénaïs Boucly, Xavier Jaïs, Laurent Savale, Olivier Sitbon, Laurent Bertoletti, Marc Humbert, David Montani","doi":"10.1183/23120541.01606-2025","DOIUrl":"10.1183/23120541.01606-2025","url":null,"abstract":"<p><strong>Rationale: </strong>Severe pulmonary hypertension (PH) is a rare clinically significant complication of COPD. The prognostic relevance of acute pulmonary vasoreactivity is well defined in pulmonary arterial hypertension (PAH) but remains poorly understood in PH-COPD.</p><p><strong>Methods: </strong>We reported in a retrospective cohort study the clinical, functional and haemodynamic characteristics, the response to PAH-approved drugs, and the overall survival of patients with severe PH-COPD from the French PH National Referral Centre, according to acute pulmonary vasoreactivity testing. The acute vasodilator response was defined as the per cent decrease in pulmonary vascular resistance (PVR) during nitric oxide (NO) testing (ΔPVR post-NO).</p><p><strong>Results: </strong>42 patients (female/male ratio 0.2, aged 65 (59-71) years) with severe PH-COPD (PVR 7.4 (6.2-9.3) WU, forced expiratory volume in 1 s 61 (50-84)%, diffusing capacity of the lung for carbon monoxide (<i>D</i> <sub>LCO</sub>) 24 (18-38)%) underwent NO testing at diagnosis. During NO testing, PVR decreased significantly from 7.4 (5.1-9.3) to 6.1 (5.0-8.5) WU (p<0.0001). An acute NO response (ΔPVR post-NO ≥19%) was associated with improved survival at 1, 3 and 5 years (93%, 70% and 61%) compared with patients with ΔPVR post-NO <19% (44%, 38% and 19%; p=0.01). Amongst 28 patients receiving PAH-approved drugs, ΔPVR post-NO correlated with PVR change at first reassessment.</p><p><strong>Conclusion: </strong>In severe PH-COPD, acute pulmonary vasoreactivity is associated with improved long-term survival and a better response to PAH-approved therapies. Further studies are warranted to confirm that acute NO testing may help identify a specific PH-COPD subgroup likely to benefit from PAH-approved treatment.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617601/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073801","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.00328-2026
Linh T Ngo, Erik Way, Caroline Muegge, Ariel Barnhardt, Michaella J Rekowski, Bryant R England, Paul J Wolters, Philip L Molyneaux, Joyce S Lee, Joshua J Solomon, Koji Kuronuma, Michael P Washburn, Scott M Matson
{"title":"Lung microenvironment proteomics identifies immune protein expression in interstitial pneumonia with autoimmune features.","authors":"Linh T Ngo, Erik Way, Caroline Muegge, Ariel Barnhardt, Michaella J Rekowski, Bryant R England, Paul J Wolters, Philip L Molyneaux, Joyce S Lee, Joshua J Solomon, Koji Kuronuma, Michael P Washburn, Scott M Matson","doi":"10.1183/23120541.00328-2026","DOIUrl":"10.1183/23120541.00328-2026","url":null,"abstract":"<p><p><b>This study characterised differential protein expression using label-free proteomics of the lung microenvironment using bronchoalveolar lavage fluid and identified increased immune protein expression in the IPAF lung compared to IPF</b> https://bit.ly/4edhp3B.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617604/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073791","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.00361-2026
Brenda O'Neill, Rohan Anand, Jeremy S Brown, Frances Butler, Lorcan McGarvey, Cecilia M O'Kane, Mike Clarke, Danny F McAuley, Adam T Hill, Anthony De Soyza, James D Chalmers, Andrew Jackson, Ben McCullough, Fiona Copeland, J Stuart Elborn, Judy M Bradley
{"title":"Adjudication of exacerbations in bronchiectasis using modified EMBARC criteria in the CLEAR Trial.","authors":"Brenda O'Neill, Rohan Anand, Jeremy S Brown, Frances Butler, Lorcan McGarvey, Cecilia M O'Kane, Mike Clarke, Danny F McAuley, Adam T Hill, Anthony De Soyza, James D Chalmers, Andrew Jackson, Ben McCullough, Fiona Copeland, J Stuart Elborn, Judy M Bradley","doi":"10.1183/23120541.00361-2026","DOIUrl":"10.1183/23120541.00361-2026","url":null,"abstract":"<p><strong>Introduction: </strong>The EMBARC consensus criteria can be used to identify and categorise pulmonary exacerbations (PEs) in clinical trials. This paper describes methods used to independently adjudicate PEs using modified EMBARC criteria in a large multicentre bronchiectasis trial (CLEAR ISRCTN89040295).</p><p><strong>Methods: </strong>The Respiratory and Systemic Symptoms Questionnaire was used to objectively collect signs and symptoms at the onset of a PE. A mEMBARC PE was defined as ≥3 symptoms, with symptoms present for ≥48 h and prescription of an antibiotic. Individual sites recorded antibiotic use for PEs. An adjudication panel independently adjudicated and then agreed on PEs according to mEMBARC criteria. Data required to determine PEs using modified Fuchs criteria was also collected.</p><p><strong>Results: </strong>Sites reported 462 PEs from 203 patients reported in the CLEAR trial. The adjudication panel categorised these exacerbations as: 239 out of 462 (52%) fully qualifying mEMBARC PEs (meeting all mEMBARC criteria), 32 out of 462 (7%) partially qualifying mEMBARC PEs (≤3 symptoms present for ≥48 h or ≥3 symptoms present for <48 h and antibiotics prescribed) and 191 out of 462 (41%) non-mEMBARC PEs (antibiotics prescribed but symptoms and duration not met). When data were classified using modified Fuchs criteria a sensitivity analysis produced results that were consistent with the main trial results.</p><p><strong>Conclusion: </strong>PEs are increasingly used as the primary outcome in bronchiectasis trials, making accuracy in their identification essential. More rigorous capture of exacerbation symptoms and implementation of a standardised and reproducible decision-making process, supported by an independent adjudication panel, is important for capturing PEs and ensuring the integrity of trial outcomes.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617598/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073768","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.00283-2026
Paula Johanna Filser, Pauline Sarah Münchenberg, Elke Berger, Reinhard Busse, Carmen Garcia, Tobias Kurth
{"title":"Clinical and economic effectiveness of telehealth in home mechanical ventilation.","authors":"Paula Johanna Filser, Pauline Sarah Münchenberg, Elke Berger, Reinhard Busse, Carmen Garcia, Tobias Kurth","doi":"10.1183/23120541.00283-2026","DOIUrl":"10.1183/23120541.00283-2026","url":null,"abstract":"<p><strong>Background: </strong>The number of patients requiring home mechanical ventilation is rising, while in-person follow-up is increasingly burdensome for patients and health systems. Telehealth may maintain clinical stability and improve care efficiency. This systematic review examined the clinical and economic effectiveness of telehealth in the management of home mechanical ventilation.</p><p><strong>Methods: </strong>The study protocol was registered <i>a priori</i> in PROSPERO. A systematic search was conducted in MEDLINE, Embase and Web of Science. The reporting of the systematic review was aligned with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines. We included quantitative studies comparing telehealth-supported home ventilation with standard care, reporting clinical or economic outcomes.</p><p><strong>Results: </strong>From 356 identified studies, 11 met the inclusion criteria: nine randomised controlled trials, one cohort study and one retrospective analysis, mostly conducted in Europe, with a total of 1458 participants. The study populations included patients with, <i>e.g.</i> COPD, amyotrophic lateral sclerosis and obesity hypoventilation syndrome. Interventions involved telehealth-supported home ventilation management. Across studies, telehealth-supported care achieved clinical outcomes comparable to those of standard care. Arterial carbon dioxide tension (<i>P</i> <sub>aCO<sub>2</sub></sub> ) control and health-related quality of life were similar between groups, with one study reporting greater <i>P</i> <sub>aCO<sub>2</sub></sub> reduction with telemonitoring. Evidence on the number of hospitalisations and exacerbations was mixed. Mortality and survival rates showed nonsignificant trends towards benefit; one cohort study reported improved first-year survival. Economic outcomes varied: home initiation reduced costs, while long-term follow-up results were heterogeneous.</p><p><strong>Conclusions: </strong>elehealth-supported home ventilation appears to be a comparable alternative to in-hospital management and often provides economic advantages. Future research should focus on standardised outcome measures, longer follow-up and health-system-specific cost-effectiveness analyses to guide sustainable implementation.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617588/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073778","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.00116-2026
Matthew J Martin, Ian D Pavord, James D Chalmers, Dinesh Saralaya, Kay G Roy, Manish Patel, Graham W Clarke, Laura Belton, Annika Rutgersson, Per Gustafson, Vieshal Raja Gopal, Tim Harrison
{"title":"Airway inflammation and asthma control in patients using salbutamol or budesonide/formoterol reliever: a randomised, controlled study.","authors":"Matthew J Martin, Ian D Pavord, James D Chalmers, Dinesh Saralaya, Kay G Roy, Manish Patel, Graham W Clarke, Laura Belton, Annika Rutgersson, Per Gustafson, Vieshal Raja Gopal, Tim Harrison","doi":"10.1183/23120541.00116-2026","DOIUrl":"10.1183/23120541.00116-2026","url":null,"abstract":"<p><strong>Background: </strong>Maintenance and reliever therapy (MART) with budesonide/formoterol (BUD/FORM) may offer asthma control with a single inhaler. This 6-month exploratory study monitored asthma symptoms, reliever use, lung function, airway inflammation and exacerbation events to determine treatment effectiveness in patients receiving BUD/FORM MART or BUD/FORM maintenance therapy plus short-acting β<sub>2</sub>-agonist (SABA).</p><p><strong>Methods: </strong>In the open-label STIFLE study (NCT03924635), patients were randomised to BUD/FORM MART (100 μg/6 μg or 200 μg/6 μg per inhalation, <i>pro re nata</i> (PRN)) or the same dose of BUD/FORM BID as maintenance therapy plus SABA (salbutamol) reliever PRN (100 μg per inhalation). Data were captured at home each day using devices connected to a smartphone <i>via</i> the STIFLE application. Occurrence of pre-defined exacerbation events required patients to attend hospital for assessment.</p><p><strong>Results: </strong>42 patients were randomised. Adherence to daily measurements exceeded 80% in both treatment groups. The proportion of patients experiencing an exacerbation was 27.8% in the BUD/FORM MART <i>versus</i> 70.8% in the BUD/FORM maintenance plus SABA group. A similar trend was observed in patients who experienced ≥1 day with ≥6 reliever uses (3 (16.7%) <i>versus</i> 9 (37.5%) patients, respectively). Patients treated with BUD/FORM MART showed less variation in airway inflammation, asthma symptom scores, lung function parameters and reliever use, <i>versus</i> BUD/FORM maintenance therapy plus SABA. No unexpected safety findings were observed.</p><p><strong>Conclusions: </strong>Patients with moderate asthma treated with BUD/FORM MART recorded less variation in asthma outcomes and lower rates of exacerbation events than those treated with BUD/FORM maintenance therapy plus SABA, suggesting improved asthma control.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617593/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073803","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
ERJ Open ResearchPub Date : 2026-09-28eCollection Date: 2026-09-01DOI: 10.1183/23120541.00049-2026
Célia Petitjean, Edouard Ollier, Pierre Labeix, Isabelle Court-Fortune, Frédéric Roche
{"title":"Effect of ventilatory support pressure during exercise on endurance time in patients with COPD: a systematic review.","authors":"Célia Petitjean, Edouard Ollier, Pierre Labeix, Isabelle Court-Fortune, Frédéric Roche","doi":"10.1183/23120541.00049-2026","DOIUrl":"10.1183/23120541.00049-2026","url":null,"abstract":"<p><strong>Rationale: </strong>Noninvasive ventilation during exercise has been proposed to improve exercise tolerance in patients with COPD. However, the influence of fixed pressure support intensity on endurance performance and exercise-related physiological responses remains insufficiently clarified.</p><p><strong>Materials and methods: </strong>A systematic review and meta-analysis were conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines (PROSPERO CRD420251018584). Randomised controlled and crossover trials evaluating noninvasive ventilation delivered in fixed pressure support mode during exercise were included. Random-effects models were applied to pool standardised mean differences (SMD). Mixed-effects meta-regression analyses assessed the association between pressure support level and outcomes. The primary outcome was endurance time. Secondary outcomes included dyspnoea (modified Borg scale) and hypercapnia.</p><p><strong>Results: </strong>A total of 15 studies (285 participants) were included. Noninvasive ventilation significantly improved endurance time compared with control conditions (SMD 0.47, 95% CI 0.20 to 0.73). Pressure support intensity was not significantly associated with endurance time (β=0.0345 per cmH<sub>2</sub>O, p=0.25). Dyspnoea was significantly reduced in pooled analysis (SMD -0.50, 95% CI -0.92 to -0.08) and showed a significant dose-response relationship with pressure support (β= -0.0880 per cmH<sub>2</sub>O, p=0.019). Carbon dioxide tension was significantly reduced with noninvasive ventilation (SMD -0.48, 95% CI -0.92 to -0.04, I<sup>2</sup>=68.2%), with a pressure-dependent reduction observed when post-exercise measurements were excluded (β= -0.1055 per cmH<sub>2</sub>O, p=0.033).</p><p><strong>Conclusion: </strong>Noninvasive ventilation during exercise improves endurance and dyspnoea in COPD. Pressure support intensity influences ventilatory unloading, but endurance performance appears multifactorial. Further standardised studies are needed to optimise pressure titration during exercise.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13617614/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149073811","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}