ERJ Open Research最新文献

{"title":"Managing multimorbidity in severe asthma: comparison of resources and perspectives between severe asthma specialists and general respiratory physicians across Europe.","authors":"Dace Matīsa, Aruna T Bansal, Saša Rink, Anna Freeman, Betty Frankemölle, Mehar Singh, Jacob K Sont, Apostolos Bossios, Ben Ainsworth, Michael Hyland, Rekha Chaudhuri, Florin Mihaltan, Antonio Spanevello, Enrico Heffler, Ian Adcock, Martina Zappa, Giorgio Walter Canonica, Guy Brusselle, Arnaud Bourdin, Giulia Anna Maria Luigia Costanzo, Ildiko Horvath, Dóra Lúðvíksdóttir, Stefania Principe, Peter Kopač, Cláudia Chaves Loureiro, Arne Egesten, Stephanie Korn, Konstantinos Samitas, Marcus Butler, Joerg Leuppi, Edin Jusufovic, Felix Wantke, Shane Hanon, Fabienne Jaun, Virginija Kalinauskaite-Zukauske, Sanja Dimic-Janjic, Graham Roberts, Sanja Hromis, Branislava Milenkovic, Judit Varkonyi-Sepp, Ozlem Goksel, Ana Margarida Pereira, Ratko Djukanovic, Angela Rizzi, Marco Caminati, Ruihua Hou, Anamarija Štajduhar, Dóra Paróczai, Luisa Brussino, Liam G Heaney, Hans Michael Haitchi, Matteo Bonnini, Kristina Bieksiene, Ebru Damadoglu, Valentyna Yasinska, Bilun Gemicioglu, Sanja Popović Grle, Anneke Ten Brinke, Zsuzsanna Csoma, Iveta Kroiča, Piotr Kuna, Celeste Porsbjerg, Hilary Hodge, Florence Schleich, Sabina Skrgat, Ramesh J Kurukulaaratchy","doi":"10.1183/23120541.00157-2026","DOIUrl":"10.1183/23120541.00157-2026","url":null,"abstract":"<p><strong>Background: </strong>Multimorbidity refers to the presence of multiple coexisting conditions, but is often underappreciated in the context of severe asthma (SA) management. We sought to identify differences in approaches to multimorbidity management in SA, variability in access to multidisciplinary team (MDT) resources, and whether physician perspectives on multimorbidity differ between SA specialists and general respiratory physicians.</p><p><strong>Methods: </strong>The Severe Heterogeneous Asthma Registry, Patient-centred (SHARP) Clinical Research Collaboration circulated an online physician survey <i>via</i> European national respiratory societies to assess 1) available resources to address multimorbidity and 2) physician perspectives on multimorbidity in SA.</p><p><strong>Results: </strong>495 responses from 25 European countries included 48% SA specialists and 52% general respiratory physicians. SA specialists had more experience with SA patients (20% were seeing >60 patients per month) compared to general respiratory physicians. SA specialists had greater access to multidisciplinary care - including better access to MDTs, allied health professionals and referrals to external specialists, and therefore more routinely assessed comorbidities and considered them greater influences on their practice. They also considered multimorbidity to a greater degree and rated its impact on their patients' asthma outcomes (and general health outcomes) as more substantial.</p><p><strong>Conclusions: </strong>Alongside more experience of treating SA, SA specialists have increased awareness of multimorbidity and better resources to manage it. However, access to MDTs remains a significant gap for both SA specialists and general respiratory physicians. Furthermore, both groups identified a high need for further education and training about multimorbidity. These findings highlight key areas for improvement in clinical practice, resources and training.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531303/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873302","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}

{"title":"Patient, family member and healthcare professionals' experiences of physiotherapy airway clearance techniques during hospital admission: a scoping review.","authors":"Kirsty Jerrard, Rhian Dowding, Brenda O'Neill, Judy M Bradley, Bronwen Connolly","doi":"10.1183/23120541.00140-2026","DOIUrl":"10.1183/23120541.00140-2026","url":null,"abstract":"<p><strong>Introduction: </strong>Effective secretion management is crucial for hospitalised patients with retained secretions due to critical illness, chronic respiratory disease or infection. Airway clearance techniques (ACT), primarily physiotherapist-delivered and advocated in guidelines, support secretion removal. However, limited knowledge exists regarding the experiences of patients, family members and healthcare professionals involved in ACT. The aim of the present study was to explore the experiences of patients, family members and healthcare professionals of ACT in hospital settings.</p><p><strong>Methods: </strong>Following JBI (formerly Joanna Briggs Institute) recommendations and the Preferred Reporting Items for Systematic reviews and Meta-Analyses extension for Scoping Reviews Checklist, searches were conducted in CINAHL Complete, EMBASE, MEDLINE ALL, PsycINFO and ProQuest Dissertations & Theses Global, from inception to 17 November 2025. Qualitative studies involving patients (≥16 years) receiving ACT, family members involved or healthcare professionals delivering ACT in the hospital setting were eligible. Screening and data charting were performed independently and in duplicate. Findings were synthesised descriptively and narratively.</p><p><strong>Results: </strong>Of 10 370 records identified, 11 studies met the inclusion criteria. Six studies reported primary themes related to ACT, while relevant source text from the remaining five studies was extracted and integrated into the narrative synthesis. Populations included healthcare professionals (physiotherapists, nurses, intensivists, internal medicine specialists; n=293) and patients (n=80). No studies included family members. Settings were predominantly intensive care units (ICU) (n=9), with two studies spanning ICU and ward settings. Four overarching themes emerged: three shared by patients and healthcare professionals (Knowledge, resources, and skills; Decision-making process; Perceived effects and outcomes), and one unique to healthcare professionals (Organisational factors).</p><p><strong>Conclusions: </strong>Current evidence on experiences of ACT is dominated by physiotherapists and ICU settings, with no studies capturing family perspectives and limited patient or wider multidisciplinary input across broader hospital contexts. More inclusive, stakeholder-informed research is needed to fully understand ACT use, impact and burden in clinical practice.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531308/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873355","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}

{"title":"Clinical comorbidity phenotypes in COPD and their biomarker signatures.","authors":"Line Egerod, Diana J Leeming, Morten A Karsdal, Julie C Yates, Jannie M B Sand, Cecilie L Bager","doi":"10.1183/23120541.00127-2026","DOIUrl":"10.1183/23120541.00127-2026","url":null,"abstract":"<p><p><b>Biomarkers of inflammation, coagulation and fibroblast activity differ across COPD comorbidity phenotypes, revealing biologically plausible signatures and suggesting potential endotypes that reflect subclinical disease processes</b> https://bit.ly/49t5nkW.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531309/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873114","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}

{"title":"Eosinophil biology in COPD: from scientific insight to clinical treatment action.","authors":"Steven P Cass, Mona Bafadhel","doi":"10.1183/23120541.01301-2025","DOIUrl":"10.1183/23120541.01301-2025","url":null,"abstract":"<p><p>COPD is a common lung disease that causes significant global morbidity. Strikingly, elevated blood and/or airway eosinophil levels are observed in up to 40% of COPD patients. These innate immune cells are clinically associated with increased exacerbation frequency and risk. Eosinophils are key in COPD symptom management and a drive to uncover their related immunopathology is critical to our understanding of disease progression. Recent biologic treatments targeting eosinophils have had success in reducing symptoms in eosinophilic COPD patients. However, the underlying pathways that lead to these improvements is incompletely understood. Given eosinophils are important in pathogen clearance, mucus viscosity, barrier integrity and tissue remodelling, any disease modifying treatments will have several proposed mechanisms. In this narrative review, we summarise the developmental, recruitment, activation and related pathology of eosinophils in the context of eosinophil-depleting treatments to elucidate treatment efficacy. Furthermore, we highlight potential future biological pathways for therapeutics aimed at specific eosinophil mechanisms.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531299/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873067","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}

{"title":"Using hyperpolarised xenon magnetic resonance imaging to explore breathlessness in long COVID: results from the EXPLAIN study.","authors":"Kher Lik Ng, Laura Saunders, Guilhem Collier, James Grist, Nina Kainth, Victoria Harris, Laurie Smith, Scarlett Strickland, Lotta Gustafsson, Paul Hughes, Ionitiana Evans, Leizl Alvarez, Avianna Laws, Siby Thomas, Alberto Biancardi, Jennifer Rodgers, Samantha Jones, Demi Jakymelen, Martin Brook, Thomas Newman, Megan Plowright, Lily Dryhurst-Pearce, Amany Elbehairy, Kenneth Jacob, Anthony McIntyre, David Capener, Jonathan Bray, Marianne Durrant, Kylie Yeung, Huw Walters, Violet Matthews, Lisa Watson, Gavin Vuddamalay, Gabriele Abu-Eid, Viktorie Madhusudhan Stisova, Mark Cox, Maja Lachut, Nina Mulvey, William Hickes, Alexander Horsley, Helen Davies, Alfred A Roger Thompson, Fergus Gleeson, Jim Wild, Emily Fraser","doi":"10.1183/23120541.00040-2026","DOIUrl":"10.1183/23120541.00040-2026","url":null,"abstract":"<p><strong>Background: </strong>Breathlessness is a common symptom in long COVID (LC). Using hyperpolarised xenon magnetic resonance imaging (¹²⁹Xe-MRI), we assessed whether this symptom could be attributed to abnormalities in the alveolar-capillary membrane not detected by standard investigations. We focused on never-hospitalised individuals without an identified cause for breathlessness.</p><p><strong>Methods: </strong>In this prospective, multicentre study, we compared ¹²⁹Xe-MRI, lung function, exercise capacity and symptom questionnaires in LC patients with breathlessness (BLC) to those without breathlessness (NBLC) and healthy controls. Primary outcome was whether BLC demonstrated measurable impairments in gas exchange focusing on dissolved-phase ¹²⁹Xe-MRI metrics: red blood cell to membrane ratio (RBC:M) and red blood cell to gas ratio (RBC:Gas). We also explored associations between symptoms and physiological measures.</p><p><strong>Results: </strong>Of 269 participants recruited, 196 were included in the analysis (109 BLC, 43 NBLC, 44 controls), with age and sex well matched across groups. BLC had a significantly longer interval from infection to MRI (median 632 days; p<0.001). No significant differences in global or regional RBC:M or RBC:Gas were observed across groups. BLC showed lower forced expiratory volume in 1 s, forced vital capacity, transfer factor of the lung for carbon monoxide (<i>T</i> _L _CO), and carbon monoxide transfer coefficient (<i>K</i> _CO) z-scores compared to controls, though >90% of values remained within normal range. A subset of BLC participants with low <i>T</i> _L _CO (14 out of 109) showed reduced ¹²⁹Xe-MRI metrics and higher breathlessness scores.</p><p><strong>Interpretation: </strong>Most nonhospitalised LC participants exhibited no detectable pulmonary abnormalities, including those with breathlessness. However, ∼13% of breathless individuals demonstrated minor reductions in <i>T</i> _L _CO and ¹²⁹Xe-MRI gas exchange suggesting a potential pulmonary contribution for symptoms in this subgroup.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531307/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873347","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}

{"title":"Proteome-wide Mendelian randomisation of lung function to identify potential therapeutic targets for respiratory disease.","authors":"Jing Chen, Nick Shrine, Kayesha Coley, Richard J Packer, Ahmed Edris, Abril G Izquierdo, Brandon Lim, Mikyeong Lee, Frank Dudbridge, Robin G Walters, Ian P Hall, Louise V Wain, Martin D Tobin, Anna L Guyatt","doi":"10.1183/23120541.00828-2025","DOIUrl":"10.1183/23120541.00828-2025","url":null,"abstract":"<p><strong>Background: </strong>Despite multiple clinical trials, disease-modifying treatments for COPD are currently limited. Since many drugs target proteins, identifying causality between proteins and lung function informs understanding of COPD pathophysiology and may suggest novel targets. We used Mendelian randomisation (MR) to prioritise proteins as potentially causal for imparied lung function. For prioritised proteins, we explored their potential suitability as drug targets by predicting their effects on a range of clinical outcomes.</p><p><strong>Methods: </strong>We used genome-wide association study (GWAS) data on 2923 proteins (n=48 195, UK Biobank) to identify single genetic variants (protein quantitative trait loci (cis-pQTLs)) associated with protein levels (p≤5×10^-9, variant ≤100 kb of a transcription start site). We performed cis-pQTL-MR analyses of four spirometric traits (n=149 166, 36 independent cohorts). Sensitivity analyses included colocalisation and reverse direction MR. We report associations between cis-pQTLs for prioritised proteins and multiple clinical respiratory outcomes, and use phenome-wide analysis to explore potential adverse effects or drug repurposing opportunities.</p><p><strong>Findings: </strong>1841 proteins had a suitable cis-pQTL. We implicated 16 proteins as potentially causal for lung function (p<1.71×10^-5): seven proteins have not been implicated by previous lung function GWAS or MR (CCND2, DTD1, PILRA, PTPRK, TDRKH, GRHPR, NUDT5), and we provide corroborative evidence for 10 proteins. We add to the literature identifying surfactant protein D (SFTPD) as a candidate, yet predict that integrin subunit alpha V (ITGAV) inhibition could impair some lung function measures, mimicking adverse results from a recent trial.</p><p><strong>Interpretation: </strong>Our approach identifies proteins (some novel) that are potentially therapeutic targets for respiratory disease, and which warrant follow-up for utility and safety.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531259/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873435","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}

{"title":"Prevalence of type 2 multimorbidities in COPD compared to asthma and relationship to blood eosinophilic counts.","authors":"David M G Halpin, Jaspreet Kaur, Heath Heatley, Freya Tyrer, Cono Ariti, Rachel Pullen, Shelly Pathak, Victoria Carter, David Price","doi":"10.1183/23120541.00252-2026","DOIUrl":"10.1183/23120541.00252-2026","url":null,"abstract":"<p><strong>Background: </strong>Type 2 (T2) inflammation is increasingly recognised in COPD, though its immunopathological profile may differ from asthma. T2 inflammatory diseases often coexist, but their prevalence in COPD patients without asthma remains underexplored. This study investigated the prevalence of T2 inflammatory multimorbidities in individuals with COPD (without asthma) and compared it with those in people with asthma. It also examined the relationship between these multimorbidities and blood eosinophil counts (BEC).</p><p><strong>Methods: </strong>Using data from the Optimum Patient Care Research Database (OPCRD), we identified patients with COPD or asthma and assessed lifetime and adjusted prevalence of T2 comorbidities including allergic rhinitis, atopic dermatitis/eczema, nasal polyps, chronic rhinitis with or without polyps, eosinophilic oesophagitis and ulcerative colitis. Prevalence was stratified by BEC and adjusted for demographic and clinical variables.</p><p><strong>Results: </strong>Among 122 509 COPD and 1 154 094 asthma patients, T2 multimorbidities were present in both groups but significantly less common in COPD. Allergic rhinitis and nasal polyps were notably less prevalent in COPD, even at higher BEC levels. Prevalence of multimorbidities increased with BEC in both groups, with stronger associations observed for nasal polyps and ulcerative colitis. Eosinophilic oesophagitis was rare across both cohorts.</p><p><strong>Conclusion: </strong>T2 multimorbidities are present in COPD and their prevalence increases with BEC, though they are generally less prevalent than in asthma, suggesting distinct immunopathology.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531282/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873368","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}

{"title":"Genome-wide association study of asthma with high treatment burden and/or worse outcomes defined using electronic healthcare data in UK Biobank.","authors":"Noemi N Piga, Michael A Portelli, Nick Shrine, Jing Chen, Richard Packer, Kayesha Coley, Alexander T Williams, Chiara Batini, Catherine John, Sharon M Lutz, Kirsten R Voorhies, Albert M Levin, Nazanin Zounemat-Kermani, James E Gern, Diane R Gold, Tina V Hartert, Daniel J Jackson, Christine C Johnson, Gurjit K Khurana Hershey, Rachel L Miller, Christine M Seroogy, Edward M Zoratti, Don D Sin, Maarten van den Berge, Yohan Bossé, Robert J Hall, Dominick Shaw, Zara E K Pogson, Andrew Fogarty, Liam G Heaney, Adel H Mansur, Rekha Chaudhuri, Neil C Thomson, John W Holloway, Kian Fan Chung, John D Blakey, Louise V Wain, Carole Ober, Ann C Wu, Ian M Adcock, Ian P Hall, Christopher E Brightling, Glenda Lassi, Ian Sayers, Katherine A Fawcett","doi":"10.1183/23120541.00327-2026","DOIUrl":"10.1183/23120541.00327-2026","url":null,"abstract":"<p><strong>Background: </strong>In ∼10% of asthma patients, symptoms remain uncontrolled despite maximal treatment, representing an unmet clinical need. The causal variants, genes and pathways underlying genetic risk factors have not been fully elucidated, and it is unclear whether there are unique genetic risk factors for this asthma subtype.</p><p><strong>Methods: </strong>We used electronic healthcare records linked to UK Biobank to identify asthma patients with high treatment burden and/or worse outcomes. We performed a genome-wide association study (GWAS) with this case population and healthy controls. We sought replication for associated (p≤5×10^-6) signals in four independent studies (12 152 cases and 32 316 controls). Replicated signals were fine-mapped and linked to genes and pathways.</p><p><strong>Results: </strong>In total, 7681 participants met our case definition and showed enrichment for adult-onset asthma, female gender and higher body mass index compared to asthma individuals not meeting case criteria. GWAS with 7681 cases and 38 405 controls revealed 21 reproducible association signals that had previously been associated with asthma, but had a larger effect size in our study. Variant-to-gene mapping highlighted 85 candidate genes, five of which were considered high confidence (<i>BACH2</i>, <i>D2HGDH</i>, <i>IL1RL1</i>, <i>RPS26</i>, <i>SMAD3</i>).</p><p><strong>Conclusion: </strong>We present the first use of electronic healthcare records in UK Biobank to identify a subtype of asthma enriched for patients with high treatment burden and/or worse outcomes. Our findings support the role of known asthma genes, highlighting genetic risk variants with stronger effect in these groups of patients. The prioritised genes provide potential therapeutic opportunities for this difficult-to-treat patient population.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531276/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873291","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}

{"title":"SOLAR: a noninterventional study of outcomes over the long term of people with cystic fibrosis (CF) aged ≥6 years treated with elexacaftor/tezacaftor/ivacaftor using data from the French CF Registry.","authors":"Conor Daly, Pierre-Régis Burgel, Isabelle Sermet-Gaudelus, Carl A Baxter, Catherine Payen-Champenois, Olivier Giraudier, Heike Wohling, Pia Clara Pafundi, Antoine Bessou, Gabriela Vega-Hernandez","doi":"10.1183/23120541.00011-2026","DOIUrl":"10.1183/23120541.00011-2026","url":null,"abstract":"<p><strong>Introduction: </strong>Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has demonstrated efficacy in people with cystic fibrosis (pwCF) aged ≥6 years with ≥1 <i>F508del</i> allele in clinical trials. This registry-based cohort study aimed to describe real-world use and outcomes up to 36 months post-treatment initiation among pwCF aged ≥6 years treated with ELX/TEZ/IVA in France.</p><p><strong>Methods: </strong>Outcomes included per cent predicted forced expiratory volume in 1 s (ppFEV₁), intravenous-treated pulmonary exacerbations (PEx), hospitalisations, nutrition, lung transplantation, mortality and, among pwCF ≥18 years, quality of life (CFQ-R). All results are presented by population (≥12 years and 6-11 years). In the ≥12 years cohort, a subgroup analysis of pwCF with advanced lung disease (ALD), defined as ppFEV₁ <40% at baseline, is presented.</p><p><strong>Results: </strong>The study included 553 pwCF aged 6-11 years, with a mean±sd age of 8.8±1.9 years at baseline, ppFEV₁ of 91.3±17.6% and mean follow-up of 8.9 months. In addition, 3313 pwCF aged ≥12 years were included, with a mean±sd age at baseline of 27.6±11.7 years, ppFEV₁ of 65.4±24.8% and mean follow-up of 21.2 months. In the cohort aged 6-11 years, ppFEV₁ improved by +9.6% (95% CI: 7.6-11.6) at 12 months and <i>i.v.</i>-treated PEx annual rate decreased from 0.15 (95% CI: 0.11-0.18) to 0.02 (0.00-0.04) after 0 to 12 months post-ELX/TEZ/IVA initiation. Among pwCF ≥12 years ppFEV₁ improved post-ELX/TEZ/IVA initiation by +16.2% (95% CI: 15.6-16.8) at 24 months. <i>i.v.</i>-treated PEx annual rate decreased from 0.92 (95% CI: 0.88-0.95) during baseline to 0.14 (95% CI: 0.12-0.15) after 12 to 24 months post-ELX/TEZ/IVA initiation. Similar benefits to those in the ≥12 years population were observed in the ALD subgroup.</p><p><strong>Conclusion: </strong>This study confirms the longer term, transformational clinical benefit of ELX/TEZ/IVA in pwCF aged ≥6 years in a French population, demonstrating sustained improvements across multiple health outcomes, including in pwCF with ALD that were not part of the pivotal clinical trials.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531278/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873354","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}

{"title":"Outcomes of transition from selexipag to parenteral prostacyclin analogues in patients with pulmonary arterial hypertension.","authors":"Ebrahim AlEissa, Lisa Kolkman, Ali Kapasi, Defen Peng, Steeve Provencher, Nathan Brunner, John Swiston","doi":"10.1183/23120541.00131-2026","DOIUrl":"10.1183/23120541.00131-2026","url":null,"abstract":"<p><p><b>Transitioning from selexipag to parenteral prostacyclin analogues yielded haemodynamic improvement. Outcomes are poor among those within this population who do not achieve a low-risk status after the transition.</b> https://bit.ly/4sMY3aF.</p>","PeriodicalId":11739,"journal":{"name":"ERJ Open Research","volume":"12 5","pages":""},"PeriodicalIF":4.4,"publicationDate":"2026-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13531287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148873391","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}