European Respiratory Journal最新文献

{"title":"Towards a multi-scale model of vascular remodelling in pulmonary arterial hypertension: the role of quantitative micro-CT.","authors":"Farbod N Rahaghi, Adam J Brownstein, Rajan Saggar","doi":"10.1183/13993003.01236-2026","DOIUrl":"https://doi.org/10.1183/13993003.01236-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"68 3","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148911353","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":"Distribution of blood eosinophil counts in the Chinese general population.","authors":"Peiji Yao, Lan Yang, Dan Liu, Dongyu He, Lei Li, Yifei Lin, Jin Huang, Weimin Li","doi":"10.1183/13993003.01570-2025","DOIUrl":"10.1183/13993003.01570-2025","url":null,"abstract":"<p><strong>Background: </strong>Current clinical assessments of blood eosinophil (EOS) count often overlook demographic variability, particularly in Asian populations, where large-scale data remain scarce. We aimed to define demographic-specific distributions of EOS counts and identify factors associated with elevated levels in the general Chinese population.</p><p><strong>Methods: </strong>Using a large-scale Chinese health-check population dataset comprising over 680 000 participants (2010-2023), we conducted a cross-sectional study to characterise overall and demographic-stratified distributions of blood EOS counts and construct age- and sex-specific percentile curves. We then applied multivariable regression to quantify associations between EOS counts and selected respiratory and systemic conditions, adjusting for key demographic and clinical covariates. Robustness was evaluated through sensitivity analyses.</p><p><strong>Results: </strong>EOS counts showed a right-skewed distribution (median (interquartile range) 100 (60-170) cells·μL^-1) and were consistently higher in males than females (120 (80-200) <i>versus</i> 90 (50-140) cells·μL^-1). Age- and sex-specific percentile curves defined population-based reference ranges and demonstrated a U-shaped trajectory across age. In multivariable analyses, higher EOS counts were associated with male sex, age <20 or ≥70 years, smoking and higher body mass index. EOS counts were positively associated with major respiratory conditions, including asthma and COPD, as well as selected systemic comorbidities (all p_Bonferroni<0.05). Findings were robust in sensitivity analyses.</p><p><strong>Conclusions: </strong>In this large Chinese health-check population, blood EOS counts showed substantial demographic heterogeneity. These findings support age- and sex-stratified reference ranges and may inform more context-specific thresholds for eosinophilia in clinical practice.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13550824/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147812918","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":"Right ventricular adaptation after pulmonary endarterectomy and balloon pulmonary angioplasty in chronic thromboembolic pulmonary hypertension: a comparative cardiac MRI-based study.","authors":"Tamara C Rodenburg, Satoshi Higuchi, Stine Andersen, Elisa Stilling, Natalia J Braams, Azar Kianzad, Esther J Nossent, Jurjan Aman, Morten K Jensen, Won Y Kim, Marcel A M Beijk, Rutger J Lely, Jacobus A Winkelman, Lars B Ilkjær, Frances S de Man, Anton Vonk Noordegraaf, Mads J Andersen, Asger Andersen, Lilian J Meijboom, Harm Jan Bogaard","doi":"10.1183/13993003.02314-2025","DOIUrl":"10.1183/13993003.02314-2025","url":null,"abstract":"<p><strong>Background: </strong>Chronic thromboembolic pulmonary hypertension (CTEPH) is associated with elevated right ventricular (RV) afterload and progressive RV remodelling. Balloon pulmonary angioplasty (BPA) has emerged as an established and increasingly used alternative to pulmonary endarterectomy (PEA). This study aimed to evaluate haemodynamic, functional and structural cardiac responses to PEA and BPA, with specific focus on RV reverse remodelling.</p><p><strong>Methods: </strong>In this prospective, multicentre cohort study, 93 patients with CTEPH (PEA n=50, BPA n=43) from the Netherlands and Denmark underwent cardiac magnetic resonance imaging, right heart catheterisation, exercise testing and biomarker assessment at baseline and 6 months post-intervention. Longitudinal changes were assessed with repeated-measures ANOVA. A subgroup underwent analysis at 18 months.</p><p><strong>Results: </strong>BPA patients were older (mean±sd age 68.9±10.1 <i>versus</i> 62.4±11.1 years; p<i>=</i>0.004), and used more pulmonary hypertension medication. PEA-treated patients had higher RV mass index at baseline (40.1±13.5 <i>versus</i> 32.9±12.2 g·m^-2; p=0.017), and consistently higher relative RV wall thickness at baseline and follow-up (both 0.4±0.1 <i>versus</i> 0.3±0.1; p<0.001). Both interventions improved RV afterload, function and exercise capacity (all p<0.05). Larger haemodynamic improvement after PEA (mean pulmonary artery pressure and pulmonary vascular resistance; p-interaction ≤0.001) was accompanied by more pronounced RV reverse remodelling, including lower follow-up indexed RV volumes (RV end-diastolic volume indexed 76.2±16.8 <i>versus</i> 92.2±20.2 mL·m^-2; p<0.001; and RV end-systolic volume indexed 39.0±15.6 <i>versus</i> 49.0±18.8 mL·m^-2; p=0.014).</p><p><strong>Conclusions: </strong>In this multicentre European study, both therapies resulted in significant RV reverse remodelling. PEA was associated with larger absolute reductions in indexed RV mass and volumes than BPA. However, remodelling sensitivity to unloading appeared broadly similar across treatments. Despite favourable outcomes following both treatments, our findings emphasise the need for randomised controlled studies, to optimise treatment strategies for operable and inoperable patients.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147835806","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":"Antibodies in lung transplant: innocent bystanders or drivers of lung allograft failure?","authors":"Jyotika D Prasad, Gregory I Snell","doi":"10.1183/13993003.01077-2026","DOIUrl":"https://doi.org/10.1183/13993003.01077-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"68 3","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148911289","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":"Three-dimensional micro-CT analysis of vascular remodelling and vascular pruning in pulmonary arterial hypertension.","authors":"Gitte Aerts, Lynn Willems, Birger Tielemans, Emanuele Muscogiuri, Astrid Vermaut, Laura Hardy, Pieterjan Kerckhof, Vincent Geudens, Yousry Mohamady, Xin Jin, Charlotte Hooft, Marta Zapata-Ortega, Hanne Beeckmans, Jan Van Slambrouck, Joline Michiels, Ruben Celen, Baptiste De Jonghe, Mieke Boon, Sofian Bouneb, Lieven J Dupont, Saskia Bos, Laurens J De Sadeleer, Stephanie Everaerts, Robin Vos, Laurens J Ceulemans, Dirk Van Raemdonck, Tom Verbelen, Frédéric Perros, Marion Delcroix, Rozenn Quarck, Bart M Vanaudenaerde, Laurent Godinas","doi":"10.1183/13993003.02118-2025","DOIUrl":"10.1183/13993003.02118-2025","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary arterial hypertension (PAH) is a rare, progressive pulmonary vascular disorder characterised by pulmonary arteriole remodelling leading to increased pulmonary vascular resistance (PVR). Key features of PAH are vascular pruning and plexiform lesion formation. While these features have been described histologically, a detailed quantitative analysis is lacking. In this study, we used micro-computed tomography (µCT) to provide three-dimensional quantification of pulmonary vascular alterations.</p><p><strong>Methods: </strong>We performed µCT imaging on explanted lungs from seven patients with end-stage idiopathic (n=4) or heritable (n=3) PAH and five healthy controls. Arterial lumen diameter, the number of acinar vessels and plexiform lesion distribution were quantified and correlated with clinical and haemodynamic data.</p><p><strong>Results: </strong>We observed a 50% reduction in the diameter of the acinar arterial lumen and a 61% decrease in the number of intra-acinar arterioles, confirming vascular pruning. Arterial narrowing correlated with PVR and mean pulmonary artery pressure. Heritable PAH showed a higher number of plexiform lesions than idiopathic PAH, with more distal, acinar-level localisation.</p><p><strong>Conclusions: </strong>This study provides quantitative evidence of arterial narrowing and objective loss of small intra-acinar arteries in end-stage PAH, supporting their central role in elevated PVR. Pulmonary vascular rarefaction may represent an initiating process of vascular remodelling rather than a secondary phenomenon. Heritable PAH was characterised by a higher number and more distal distribution of plexiform lesions than idiopathic PAH, which may contribute to its more severe phenotype.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147987074","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":"Bridging the airway microbiome and targeted therapy in bronchiectasis: multi-omics insights, endotypes and emerging therapies.","authors":"Christina S Thornton, Laura Schaupp, Michael M Tunney, Marcus A Mall","doi":"10.1183/13993003.00239-2026","DOIUrl":"10.1183/13993003.00239-2026","url":null,"abstract":"<p><p>Bronchiectasis is a heterogeneous chronic airway disease primarily driven by persistent infection, microbial dysbiosis and dysregulated host immunity. While culture-based microbiology has historically informed clinical management, advances in high-throughput sequencing and multi-omic technologies have transformed our understanding of the airway ecosystem, revealing that disease activity is shaped not only by individual pathogens, but by complex and dynamic host-microbe interactions. Despite the breadth of descriptive microbiome data, translation into clinically actionable diagnostics or therapies has been limited. Importantly, cross-sectional correlations between microbiota and inflammation do not establish cause and effect, underscoring the need to embed host-microbiome profiling within both longitudinal and interventional therapeutic trials. In this review, we critically appraise current microbial and host multi-omics research in bronchiectasis, integrating microbiome studies with host inflammatory, proteomic and immunophenotyping data. We highlight themes emerging across cohorts, including low microbial diversity, pathogen dominance, loss of commensal networks and neutrophil-driven inflammation, and discuss how these features align with biological endotypes associated with exacerbations and treatment response. Drawing on lessons from host-directed therapeutic successes, we examine translational roadblocks limiting microbiome-guided care. We further review emerging microbiome-modulating strategies such as pathogen-specific biologics, bacteriophage therapy, live biotherapeutic products, biofilm-targeting adjuncts and precision antibiotic stewardship. Finally, we propose a roadmap toward microbiome-informed precision medicine through harmonised methodologies, integration of host and microbial biomarkers into clinical trials, and embedding multi-omics pipelines within large international registries. Collectively, these advances have the potential to shift bronchiectasis research and clinical management towards rationally designed, precision medicine-driven therapeutic strategies.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148224101","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":"When a negative SPECT V/Q is reassuring, but the trial still leaves the hardest clinical questions unanswered.","authors":"Yuanli Yang","doi":"10.1183/13993003.00787-2026","DOIUrl":"https://doi.org/10.1183/13993003.00787-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"68 3","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148911348","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":"Mechanical power and prognosis: a physiological perspective.","authors":"Francesca Collino, Irene Steinberg, Eleonora Balzani, Ilaria Grava, Andrea Formaggio, Luigi Camporota","doi":"10.1183/13993003.00082-2026","DOIUrl":"https://doi.org/10.1183/13993003.00082-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"68 3","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148911383","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 guidelines update for the treatment of pulmonary arterial hypertension.","authors":"Gabor Kovacs, Katarina Zeder, Marc Humbert, Robin Condliffe, Ingrid Toews, Athenais Boucly, Micheala Aldred, Roberto Badagliacca, Wendy Chung, Frances De Man, Peter Dorfmüller, Pisana Ferrari, George Giannakoulas, Sergio Harari, Tom Kotsimbos, Nicole Ngai Yung Tsang, Rozenn Quarck, Antenor Rodrigues, Stephan Rosenkranz, Olivier Sitbon, Anton Vonk Noordegraaf, Lan Zhao, Marius Hoeper, Marion Delcroix","doi":"10.1183/13993003.01178-2026","DOIUrl":"https://doi.org/10.1183/13993003.01178-2026","url":null,"abstract":"<p><strong>Background: </strong>Pulmonary arterial hypertension (PAH) is a severe, progressive disease of the small pulmonary arteries. These guidelines provide updated evidence-based recommendations for the treatment of PAH focusing on the latest scientific evidence on sotatercept, an activin signalling inhibitor targeting a novel pathway for the treatment of PAH, and on the role of right heart catheterisation (RHC) in the management of PAH patients during follow-up.</p><p><strong>Methods: </strong>A European Respiratory Society (ERS) Task Force, comprising global experts, methodologists and a patient representative, developed these clinical practice guidelines in accordance with ERS methodology and the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach. Four PICO (Patients, Intervention, Comparator, Outcomes) questions and one narrative question have been addressed.</p><p><strong>Recommendations: </strong>The Task Force recommends add-on treatment with sotatercept in PAH patients who already receive PAH drugs and are at intermediate-low, intermediate-high or high risk of death during follow-up; this recommendation is based on a high certainty of evidence available from randomised controlled trials (RCTs). In PAH patients at low risk of death, the Task Force does not provide a recommendation due to the low certainty of evidence and the limited number of such patients included in RCTs. To monitor safety and efficacy, patients receiving sotatercept should be treated in pulmonary hypertension centres and their management should include regular follow-up and appropriate examinations. To guide treatment strategy, the Task Force suggests performing RHC during follow-up in PAH patients who receive PAH drugs and are at intermediate-low, intermediate-high or high risk of death, when therapeutic consequences are expected. The Task Force does not provide a recommendation for this question in PAH patients at low risk of death, due to the very low certainty of evidence.</p><p><strong>Conclusion: </strong>The ERS guidelines on the treatment of PAH provide an updated evidence-based framework for the optimal management of patients with PAH.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148905669","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":"Solving an enigma: the BMP-9/10 axis as a key regulator of pulmonary vascular resilience.","authors":"Christophe Guignabert, Marc Humbert","doi":"10.1183/13993003.00621-2026","DOIUrl":"https://doi.org/10.1183/13993003.00621-2026","url":null,"abstract":"<p><p>The circulating ligands bone morphogenetic protein (BMP)-9 and BMP-10 are emerging as dual regulators of pulmonary and systemic vascular biology. Acting through activin receptor-like kinase 1 (ALK1), BMP receptor type II (BMPRII) and endoglin receptor complexes, they maintain endothelial quiescence and vascular tone under physiological conditions. Yet, the same axis can turn pathogenic when its intensity, timing or cellular context are altered. We propose viewing BMP-9/10 not as a linear protective pathway but as a bimodal system in which both deficiency and hyperactivation disrupt vascular homeostasis, leading to distinct phenotypes such as obstructive pulmonary arterial remodeling in pulmonary arterial hypertension (PAH), intrapulmonary vasodilatation in hepatopulmonary syndrome (HPS), and arteriovenous shunting in hereditary haemorrhagic telangiectasia (HHT). The clinical success of sotatercept, an activin signaling inhibitor with partial BMP-ligand trap properties, underscores the translational potential of therapeutically \"retuning\" this pathway rather than globally enhancing it. Understanding when BMP-9/10 signaling protects and when it becomes pathogenic will be crucial for designing next-generation interventions that stabilize, instead of destabilize, pulmonary vascular integrity.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148905729","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}