European Respiratory Journal最新文献

{"title":"Combined Supplemental Oxygen and Mandibular Advancement Device Therapy for Obstructive Sleep Apnea: A Randomized-Controlled Trial.","authors":"Bradley A Edwards,Luke D J Thomson,Daniel Vena,Andrew Gikas,Reza Radmand,Nicole Calianese,Lauren B Hess,Jinny Collet,Dillon Gilbertson,Natalie V Lawrence,Shane A Landry,Simon A Joosten,Garun S Hamilton,Andrew Wellman,Scott A Sands","doi":"10.1183/13993003.01320-2025","DOIUrl":"https://doi.org/10.1183/13993003.01320-2025","url":null,"abstract":"INTRODUCTIONTreatment for obstructive sleep apnoea (OSA) is limited by intolerance to continuous positive airway pressure. While OSA manifests due to both increased pharyngeal collapsibility and ventilatory control instability, randomized trials targeting both components are lacking. We tested whether combining a ventilatory control intervention (supplemental oxygen) with an upper airway mechanical intervention (mandibular advancement devices, MAD) improves treatment efficacy.METHODSIn a multicentre randomized crossover trial, 41 patients with moderate-to-severe OSA (apnoea-hypopnoea index [AHI] >20 events/hr, scored without desaturation criteria) underwent polysomnography with four single-night interventions: oxygen (4 L·min-1), MAD, combination therapy, and sham (air). Primary analysis compared percent change in AHI from baseline between combination therapy and MAD monotherapy. Secondary outcomes included arousal index and visual analog scale for sleep quality (VASSQ). Gold-standard baseline pathophysiological traits facilitated mechanistic subgroup analysis.RESULTSOf 41 randomized patients (14F:27 M, AHIbaseline=49 [29, 62] events/hr; median [interquartile range]), 38 completed all interventions. Compared to sham, AHI was lowered with oxygen (estimate[CI]: -33[-46, -17]%), MAD (-54[-64, -41]%), and the combination (-68[-77, -57]%); the combination provided a greater reduction than MAD monotherapy (-14[-23, -4]%, p=0.009). The combination also improved AHI per 3% desaturation/arousal criteria (-73[-81, -62]% versus sham, -17[-25, -7]% versus MAD). The combination improved arousal index (-36[-43, -27]% versus sham) and VASSQ (+0.98[0.39, 1.58] versus sham), albeit not significantly beyond MAD alone. Effects were greatest in those with higher loop gain and collapsibility.CONCLUSIONCombining a ventilatory control intervention (supplemental oxygen) with an upper airway intervention (MAD) is a promising approach to markedly attenuate OSA severity beyond that offered by each intervention alone.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"10 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145209153","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"European Respiratory Society Clinical Practice Guideline for the Management of Adult Bronchiectasis.","authors":"James D Chalmers,Charles S Haworth,Patrick Flume,Merete B Long,Pierre Régis Burgel,Katerina Dimakou,Francesco Blasi,Beatriz Herrero-Cortina,Raja Dhar,Sanjay H Chotirmall,Felix C Ringshausen,Josje Altenburg,Lucy Morgan,Mattia Nigro,Megan L Crichton,Chayenne Van Meel,Oriol Sibila,Alan Timothy,Eliza Kompatsiari,Tanja Hedberg,Thomas Vandendriessche,Pamela J McShane,Thomy Tonia,Kevin Winthrop,Michael R Loebinger,Natalie Lorent,Pieter Goeminne,Michal Shteinberg,Eva Polverino,Stefano Aliberti","doi":"10.1183/13993003.01126-2025","DOIUrl":"https://doi.org/10.1183/13993003.01126-2025","url":null,"abstract":"BACKGROUNDBronchiectasis is a common lung condition associated with wide range of infectious, immunological, autoimmune, allergic and genetic conditions. Exacerbations and daily symptoms have the largest impact on patients and healthcare systems, and they are the key focus of treatments. Current practice is heterogeneous globally, and bronchiectasis has historically been a neglected disease. Here, we present evidence-based international guidelines for the management of adults with bronchiectasis.METHODSA European Respiratory Society (ERS) Task Force, comprising global experts, a methodologist, and patient representatives, developed clinical practice guidelines in accordance with ERS methodology and the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach. Systematic literature searches, data extraction, and meta-analysis were performed to generate evidence tables, and recommendations were formulated using the evidence-to-decision framework. A total of 8 PICO (Patient, Intervention, Comparator, Outcomes) questions and 3 narrative questions were developed.RECOMMENDATIONSThe Task Force recommendations include strong recommendations in favour of airway clearance techniques for most patients with bronchiectasis and pulmonary rehabilitation for those with impaired exercise capacity. We issue a strong recommendation for the use of long-term macrolide treatment for patients at high risk of exacerbations and a strong recommendation in favour of long-term inhaled antibiotics in patients with chronic Pseudomonas aeruginosa infection at high risk of exacerbation. Conditional recommendations support the use of eradication treatment or mucoactive drugs in specific circumstances. We suggest not to routinely use long term oral, non-macrolide antibiotic treatment or inhaled corticosteroids. Additional guidance is also provided on testing for underlying causes, managing exacerbations, and managing the deteriorating patient.CONCLUSIONThe ERS bronchiectasis guidelines provide an evidence-based framework for optimal management of adults with bronchiectasis and serve as a benchmark for evaluating the quality of care.SCOPE AND OBJECTIVESThe European Respiratory Society (ERS) guidelines for the management of bronchiectasis in adults provide evidence-based recommendations for the care of people with clinically significant bronchiectasis, defined by the presence of permanent dilatation of the bronchi evident on chest CT scan, along with characteristic clinical symptoms. [1] These guidelines are intended for all healthcare professionals involved in the care of adults with bronchiectasis, as well as for policymakers, regulatory authorities, and pharmaceutical companies. Bronchiectasis is a complex and heterogeneous disease; therefore, no guideline can be entirely comprehensive or replace clinical judgement. All guideline recommendations must be interpreted within the specific clinical context in which they are applied. Separate ERS guidelines","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"4 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-09-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145182550","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":"Intravenous iron for the treatment of iron deficiency in adults with cystic fibrosis: a prospective observational cohort study.","authors":"Nick P Talbot,Matilda Downs,Jennifer Cane,Sandi Yen,Goran Mohammad,Alison Gates,Joanna Snowball,Magda Laskawiec-Szkonter,Melissa Dobson,Samira Lakhal-Littleton,Jethro S Johnson,Najib M Rahman,Stephen Gerry,Stephen J Chapman,William G Flight","doi":"10.1183/13993003.00838-2025","DOIUrl":"https://doi.org/10.1183/13993003.00838-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"52 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145153512","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"European Respiratory Society and American Thoracic Society guidelines for the diagnosis of Primary Ciliary Dyskinesia.","authors":"Amelia Shoemark,Myrofora Goutaki,BreAnna Kinghorn,Cristina Ardura-Garcia,Noelia Baz-Redón,Mark Chilvers,Stephanie D Davis,Jana De Brandt,Sharon Dell,Raja Dhar,Lucy Dixon,Thomas Ferkol,Claire Hogg,Marie Legendre,Margaret Leigh,Jane S Lucas,Michele Manion,Nisreen Rumman,Ingrid Toews,Valerie Labonte,Wallace B Wee,Panayiotis Kouis,Amjad Horani","doi":"10.1183/13993003.00745-2025","DOIUrl":"https://doi.org/10.1183/13993003.00745-2025","url":null,"abstract":"Primary ciliary dyskinesia (PCD) is caused by pathogenetic variants in >55 genes. PCD is associated with early-onset chronic wet cough and rhinosinusitis, laterality defects, middle ear disease, and reduced fertility. The clinical presentation is heterogeneous, and diagnosis often relies on multiple tests. The American Thoracic Society (ATS) and European Respiratory Society (ERS) have previously developed separate guidelines for diagnosis. Here, ERS and ATS members systematically reviewed the literature on diagnostic tools used in practice and developed unified evidence-based guidelines for PCD diagnosis using GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology, and a transparent process of decision-making using Evidence-to-Decision (EtD) frameworks. The Task Force panel formulated three PICO (Patients, Intervention, Comparison, Outcomes) questions and three narrative questions. The accuracies of high-speed video microscopy (HSVM), immunofluorescence (IF), and nasal nitric oxide (nNO) were compared to a reference test of transmission electron microscopy (TEM) and/or genetics. The panel gives strong recommendation for use of HSVM, IF, and nNO as adjunct tests to TEM and/or genetics for PCD diagnosis. However, no adjunct test is suitable as a standalone test to diagnose PCD and no single adjunct or reference test is suitable to exclude PCD. Pursuing a genetic diagnosis is encouraged due to the implication on management. The panel emphasizes that tests should meet a minimum standard and proposes evaluation of patients at a referral centre experienced in diagnosis. The pretest probability based on symptoms should be considered when interpreting results.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"42 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145153515","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":"Reply to: Pulmonary vascular resistance to define severe pulmonary hypertension: cutting the Gordian knot.","authors":"Robert Naeije,Ari Chaouat,Michael R Pinsky","doi":"10.1183/13993003.01474-2025","DOIUrl":"https://doi.org/10.1183/13993003.01474-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"99 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145140049","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":"Lymphoma in patients with asthma treated with dupilumab: much ado about nothing?","authors":"Timothy J Davies,Ian D Pavord","doi":"10.1183/13993003.01321-2025","DOIUrl":"https://doi.org/10.1183/13993003.01321-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"86 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145140052","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":"Genomics of chronic dry cough unravels neurological pathways.","authors":"Kayesha Coley, Catherine John, Jonas Ghouse, David J Shepherd, Nick Shrine, Abril G Izquierdo, Stavroula Kanoni, Emma F Magavern, Richard Packer, Lorcan McGarvey, Jaclyn A Smith, Henning Bundgaard, Sisse R Ostrowski, Christian Erikstrup, Ole B V Pedersen, David A van Heel, William Hennah, Mikko Marttila, Robert C Free, Edward J Hollox, Louise V Wain, Martin D Tobin, Chiara Batini","doi":"10.1183/13993003.02341-2024","DOIUrl":"10.1183/13993003.02341-2024","url":null,"abstract":"<p><strong>Background: </strong>Chronic dry cough is a symptom of common lung conditions, can occur as a side-effect of angiotensin-converting enzyme inhibitors (ACEis), or may be unexplained. Despite the substantial health burden presented by chronic dry cough, its biological mechanisms remain unclear. We hypothesised shared genetic architecture between chronic dry cough and ACEi-induced cough and aimed to identify causal genes underlying both phenotypes.</p><p><strong>Methods: </strong>We performed multi-ancestry genome-wide association studies (GWAS) of chronic dry cough and ACEi-induced cough, and a multi-trait GWAS of both phenotypes, utilising data from five cohort studies. Chronic dry cough was defined by questionnaire responses, and ACEi-induced cough by treatment switches or clinical diagnosis in electronic health records. We mapped putative causal genes and performed phenome-wide association studies (PheWAS) of associated variants, and polygenic scores for ACEi-induced cough, to identify pleiotropic effects.</p><p><strong>Results: </strong>We found seven novel genetic association signals reaching p<5×10^-8 in the multi-trait or single-trait analyses of chronic dry cough and ACEi-induced cough. The novel variants mapped to 10 novel genes, and we mapped an additional three novel genes to known risk variants, many of which implicate neurological functions (<i>CTNNA1</i>, <i>KCNA10</i>, <i>MAPKAP1</i>, <i>OR4C12</i>, <i>OR4C13</i>, <i>SIL1</i>). The polygenic-score-based PheWAS highlighted associations with an elevated risk of several clinical conditions including asthma, diabetes and multi-site chronic pain.</p><p><strong>Conclusion: </strong>Our findings provide support for neuronal dysfunction underlying cough hypersensitivity in chronic dry cough and ACEi-induced cough, and identify diseases and traits associated with genetic predisposition to cough that could inform drug target discovery.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12461901/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144658837","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":"Rbms1 promotes pulmonary fibrosis by stabilising Sumo2 mRNA to facilitate Smad4-SUMOylation and fibroblast activation.","authors":"Yingying Guo, Qianqian Wang, Yuhan Zhang, Lingxue Ren, Yuquan Wang, Yang Liu, Miao Liu, Xiaomu Tian, Qiudi Liu, Yi Chen, Jian Sun, Tongzhu Jin, Xinyue Wang, Yanbo Wang, Tianyu Li, Yuhong Zhou, Zhixin Li, Yunyan Gu, Baofeng Yang, Haihai Liang","doi":"10.1183/13993003.01667-2024","DOIUrl":"10.1183/13993003.01667-2024","url":null,"abstract":"<p><strong>Background: </strong>The formation of myofibroblast foci constitutes a hallmark pathological feature of idiopathic pulmonary fibrosis (IPF), yet the mechanism remains elusive. RNA-binding motif single-stranded interacting protein 1 (RBMS1) is known to be essential for proliferation and cell cycle progression; however, its role in pulmonary fibrosis remains to be clarified.</p><p><strong>Methods: </strong>This study aimed to systematically elucidate the role and underlying mechanism of RBMS1 in pulmonary fibrosis utilising mouse primary lung fibroblasts (mPLFs), fibroblast-specific Rbms1 deletion and overexpression mice models, and lung samples from IPF patients.</p><p><strong>Results: </strong>RBMS1 was highly expressed in both IPF patient lungs and murine bleomycin-induced fibrotic lesions. Notably, elevated RBMS1 expression was observed in the cytoplasm of mPLFs following transforming growth factor (TGF)-β1 stimulation. Rbms1 promoted lung fibroblast activation, while knockdown of Rbms1 mitigated TGF-β1-induced fibrogenesis. <i>In vivo</i>, overexpression of Rbms1 impaired lung function and exacerbated pulmonary fibrosis, whereas fibroblast-specific deletion of Rbms1 exhibited a significant reduction in fibrosis post-bleomycin treatment. Mechanistically, Rbms1 binds to Sumo2 3' untranslated region, enhancing the mRNA stability. Furthermore, Rbms1 induced the SUMOylation of Smad4, with lysine 158 identified as a critical SUMOylation site. Meanwhile, Sumo2 knockdown alleviated the Rbms1-driven exacerbation of pulmonary fibrosis. Importantly, nortriptyline pharmacologically inhibited RBMS1 to ameliorate pulmonary fibrosis in mice.</p><p><strong>Conclusion: </strong>Collectively, our study sheds light on the regulatory role of RBMS1 in pulmonary fibrosis, highlighting its therapeutic potential for targeted antifibrotic strategies.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144233660","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":"Pulmonary vascular resistance to define severe pulmonary hypertension: cutting the Gordian knot.","authors":"Gabor Kovacs,Katarina Zeder,Karen M Olsson,Marius M Hoeper,Marc Humbert","doi":"10.1183/13993003.01418-2025","DOIUrl":"https://doi.org/10.1183/13993003.01418-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"1 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145140053","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":"Dissecting the genetics of chronic cough.","authors":"Alyn Hugh Morice","doi":"10.1183/13993003.01267-2025","DOIUrl":"https://doi.org/10.1183/13993003.01267-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"73 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-09-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145140050","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}