European Respiratory Journal最新文献

{"title":"Preserved terminal bronchiole density in PAH revealed by micro-CT imaging.","authors":"Gitte Aerts, Emanuele Muscogiuri, Laura Hardy, Pieterjan Kerckhof, Vincent Geudens, Xin Jin, Charlotte Hooft, Marta Zapata-Ortega, Stephanie Everaerts, Marion Delcroix, Rozenn Quarck, Bart M Vanaudenaerde, Laurent Godinas","doi":"10.1183/13993003.01230-2026","DOIUrl":"https://doi.org/10.1183/13993003.01230-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149037275","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":"Single-cell multi-omics show disruption of blood and airway T cells in pulmonary long COVID.","authors":"Elizabeth Guinto, I-Chih Kuo, Sameeksha Chopra, Samuel B Shin, Firoozeh V Gerayeli, Jung-Wan Yoo, Hye-Yun Park, Melina Messing, Xuan Li, Chen Xi Yang, Cassandra Gilchrist, Dina Yehia, Rachel L Eddy, Stephen Milne, Tara R Stach, Chung Y Cheung, Julia S W Yang, William Yip, Tawimas Shaipanich, Jonathon Leipsic, Kelly M McNagny, Janice M Leung, Don D Sin","doi":"10.1183/13993003.02001-2025","DOIUrl":"https://doi.org/10.1183/13993003.02001-2025","url":null,"abstract":"<p><strong>Background: </strong>Approximately 10% of individuals who recover from COVID-19 experience residual respiratory symptoms impacting their quality of life, but the mechanisms behind pulmonary long COVID (PLC) are largely unknown.</p><p><strong>Objectives: </strong>We characterized airway and circulating immune cells in patients with and without PLC.</p><p><strong>Methods: </strong>Participants were recruited and allocated into two groups: 1) PLC, defined by a St. George's Respiratory Questionnaire (SGRQ) total score of >10 at least three months following an acute SARS-CoV-2 infection with self-reported new or worsening symptoms, and 2) controls, defined by SGRQ ≤10 with or without a prior history of COVID. We performed research bronchoscopy and obtained bronchoalveolar lavage (BAL) in seven PLC patients and seven age- and sex-matched control subjects. Single-cell RNA sequencing (scRNAseq) was performed on the BAL cells. Peripheral blood mononuclear cells (PBMCs) were cryopreserved in 30 participants (17 PLC, 13 controls) for proteomic analysis. Serum was submitted for microarray detection of auto-IgG antibodies.</p><p><strong>Results: </strong>We annotated 105 836 cells using scRNAseq and found that CD4⁺ T cells were credibly increased in participants with PLC. scRNAseq revealed up-regulation of anti-viral pathways including those related to interferon signaling in T cells as well as antigen presenting cells. In PBMCs, T cells expressing both CD4 and CD8 were elevated in PLC participants. Autoantibodies targeting histone 2B and histone 3 were significantly increased in PLC participants (adj.p<0.05).</p><p><strong>Conclusions: </strong>PLC is associated with dysregulation of T cell mediated immunity, which may be related to autoimmunity. These cells represent potential novel therapeutic targets in patients suffering from PLC.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149037318","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":"The regulatory landscape in tuberculosis integrated research platforms.","authors":"Juan Espinosa-Pereiro, Francesca Saluzzo, Jesús González-Moreno, Marco Cavaleri, Norbert Hittel, Christian Lienhardt, Karin Rombouts, Ulrika Simonsson, Daniela Maria Cirillo, Adrián Sánchez-Montalvá","doi":"10.1183/13993003.00646-2026","DOIUrl":"https://doi.org/10.1183/13993003.00646-2026","url":null,"abstract":"<p><strong>Background: </strong>Tuberculosis (TB) is the infectious disease with the highest mortality burden globally. Despite a recently enriched drug pipeline, there has been limited advance in improving its treatment. Integrated research platforms (IRPs) provide a novel research framework which, by hosting adaptive platform trials, can streamline the evaluation of several new TB regimens, whilst reducing costs. However, there is no clear, evidence-based guidance on the use of IRPs for regulatory drug development in TB.</p><p><strong>Methods: </strong>This consensus statement summarizes the discussion and recommendations produced during the European Union Patient centric clinical trial platforms (EU-PEARL) project. A multidisciplinary task force reviewed the literature regarding regulatory pathways for IRP with special focus on TB and identified current gaps and needs, which were discussed by an extended panel in two consultations. Here we summarise the output of this process and propose possible ways to advance in the implementation of such platforms.</p><p><strong>Results and conclusion: </strong>IRP coordinators are advised to seek an early contact with national regulators and supranational organisms, including regulatory collaboration initiatives. To ensure regulatory acceptance, IRP must prioritize careful design, including clear decision rules for phase transitions, and the development of surrogate makers. Furthermore, data leverage and stratification across groups could enhance IRP success. These efforts will not only strengthen the scientific validity of the IRP but also enhance its regulatory acceptability and impact on global TB treatment strategies.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149037333","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":"Rapid, 24-hour, lung function improvement with rademikibart for asthma: post hoc analyses from a phase 2b randomised trial.","authors":"Michael E Wechsler, Barry Quart, Raúl Collazo","doi":"10.1183/13993003.01278-2026","DOIUrl":"https://doi.org/10.1183/13993003.01278-2026","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149037324","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":"Elexacaftor/tezacaftor/ivacaftor improves CFTR function to near-normal levels in children with cystic fibrosis.","authors":"Jasmin Berger, Yin Yu, Julian Berges, Alicia González, Virginia Rossow, Susanne Hämmerling, Rory E Morty, Stephanie Thee, Mirjam Stahl, Olaf Sommerburg, Marcus A Mall, Simon Y Graeber","doi":"10.1183/13993003.00422-2026","DOIUrl":"https://doi.org/10.1183/13993003.00422-2026","url":null,"abstract":"<p><strong>Background: </strong>The CFTR modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) improves CFTR function in adolescents and adults with cystic fibrosis (CF) carrying at least one <i>F508del</i> allele to around 40% of median CFTR activity in non-CF controls. This partial restoration of CFTR function is associated with residual mucus dysfunction, airway infection and inflammation. In clinical trials, ETI led to a larger reduction of sweat chloride concentration in children compared to adolescents and adults with CF. However, the degree of correction of CFTR function by ETI in children with CF is currently unknown. Therefore, the aim of this study was to quantify restoration of CFTR function in children with CF aged 2 to 11 years and one or two <i>F508del</i> alleles.</p><p><strong>Methods: </strong>This prospective observational multicenter study quantified restoration of CFTR function by intestinal current measurements (ICM) before and 4 months after initiation of ETI. Additionally, we performed a combined analysis of the effects of ETI in children and our previous study in adolescents and adults with CF.</p><p><strong>Results: </strong>A total of 26 children with CF and at least one <i>F508del</i> allele were enrolled in this study. ETI improved cAMP-induced chloride secretory response to a median of 89.5% (IQR, 51.4 to 125.3; p<0.001) and increased the total chloride secretory response to 99.2% (IQR, 66.3 to 144.3; p<0.001) of median CFTR activity in non-CF controls. Furthermore, we observed strong correlations of the cAMP response and the total chloride response with age (R²=0.43 and R²=0.53 respectively, both p<0.001).</p><p><strong>Conclusions: </strong>Our results show that treatment with ETI results in near-normal CFTR function measured in freshly obtained rectal biopsies in children with CF aged 2 to 11 years and at least one <i>F508del</i> allele.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":23.8,"publicationDate":"2026-09-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149037341","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":"Beyond Heritable PAH: Pulmonary Hypertension in Genetic Syndromes","authors":"Julien Grynblat,Florence Coulet,Thomas Lacoste-Palasset,Xavier Jais,Eric Austin,Dunbar Ivy,Maria-Rosa Ghigna,Antoine Beauvais,Etienne-Marie Jutant,Athénaïs Boucly,Mathilde Meot,Sophie-Guiti Malekzadeh-Milani,Marilyne Levy,Fabrice Antigny,Grégoire Ruffenach,Olivier Sitbon,Laurent Savale,Marc Humbert,Damien Bonnet,David Montani","doi":"10.1183/13993003.00808-2026","DOIUrl":"https://doi.org/10.1183/13993003.00808-2026","url":null,"abstract":"Pulmonary hypertension (PH) may complicate a broad range of genetic syndromes beyond the established spectrum of heritable pulmonary arterial hypertension. Although these conditions are individually rare, together they represent an emerging field at the crossroads of developmental biology, vascular medicine, and precision genomics. In many cases, PH may be the presenting feature or may remain unrecognized because it occurs within complex multisystem disorders involving congenital heart disease, developmental lung abnormalities, parenchymal lung disease, vascular malformations, or extra-pulmonary manifestations. Recent advances in human genetics have expanded the spectrum of genes and syndromes associated with PH, including disorders involving altered lung and vascular development, dysregulated hypoxia signaling, smooth muscle dysfunction, chromosomal abnormalities, and syndromic vasculopathies. In this review, we summarize the main genetic syndromes associated with PH and discuss their underlying mechanisms, clinical phenotypes, diagnostic clues, and therapeutic implications. We paid particular attention to conditions that illustrate the marked heterogeneity of syndromic PH such as FLNA -related disorders, neurofibromatosis type 1, Noonan syndrome, Down syndrome, Alagille syndrome, Cantú syndrome, Chuvash polycythaemia, cobalamin C deficiency, multisystemic smooth muscle dysfunction syndrome, alveolar capillary dysplasia with misalignment of pulmonary veins, and Moya Moya syndrome.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"20 1","pages":"2600808"},"PeriodicalIF":24.3,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148923249","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":"Reverse remodelling in chronic thromboembolic pulmonary hypertension: the right ventricular enigma continues.","authors":"Hakim Ghani, Joanna Pepke-Zaba","doi":"10.1183/13993003.01039-2026","DOIUrl":"https://doi.org/10.1183/13993003.01039-2026","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":"148911309","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":"Prioritising research topics identified by end-users for a successful and rapid uptake of new tuberculosis treatment regimens.","authors":"Alessandra Bellavia, Tsion Asfaw Refera, Lia D'Ambrosio, Rosella Centis, Onno Akkerman, Fatma Alyaquobi, Erlina Burhan, Anh Tuan Dinh-Xuan, Irina Felker, José-María García-García, Gina Gualano, Ananthu James, Syed Karam Shah, Yousra Kherabi, Yang Li, Christian Lienhardt, Rifat Mahfuza, Marcela Munoz-Torrico, Catherine W M Ong, Ramesh Panchagnula, Alberto Piubello, Daria Podlekareva, Emanuele Pontali, Molebogeng X Rangaka, Najiha Rasheed, Adrian Rendon, Denise Rossato Silva, Muhammad Abdul Hamid Selim, Barbara Seaworth, Marina Tadolini, Simon Tiberi, Simone Villa, Cary Amiel Villanueva, Mario C Raviglione, Giovanni Battista Migliori","doi":"10.1183/13993003.00989-2026","DOIUrl":"10.1183/13993003.00989-2026","url":null,"abstract":"","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":"148629705","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: When a negative SPECT V/Q is reassuring, but the trial still leaves the hardest clinical questions unanswered.","authors":"Romain Le Pennec, Grégoire Le Gal, Pierre-Yves Salaun","doi":"10.1183/13993003.01217-2026","DOIUrl":"https://doi.org/10.1183/13993003.01217-2026","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":"148911324","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":"Blood eosinophils: questioning what we call \"normal\".","authors":"Pearl Gupta, John D Blakey, Sanjay Ramakrishnan","doi":"10.1183/13993003.00883-2026","DOIUrl":"https://doi.org/10.1183/13993003.00883-2026","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":"148911312","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}