European Respiratory Journal最新文献

{"title":"cGAS: a novel therapeutic target in systemic sclerosis-associated interstitial lung disease.","authors":"Theodoros Karampitsakos,Jose D Herazo-Maya","doi":"10.1183/13993003.00835-2025","DOIUrl":"https://doi.org/10.1183/13993003.00835-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"12 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144802659","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 pressure increases during Acute Exacerbation in Chronic Obstructive Pulmonary Disease and clinical outcome.","authors":"Ema Rastoder,Pradeesh Sivapalan,Caroline Hedsund,Peter Kamstrup,Tor Biering-Sørensen,Maria Dons,Trine Charlotte Bistrup Petersen,Filip Soeskov Davidovski,Kristoffer Grundtvig Skaarup,Morten Sengeløv,Emil Durukan,Ditte Vesterlev,Helena Zander Wodschow,Lars Pedersen,Josefin Eklöf,Anna Kubel Vognsen,Mia Moberg,Julie Janner,Louise Lindhardt Toennesen,Hashmat S Z Bahrami,Ulrik Dixen,Jens Dahlgaard Hove,Magnus Thorsten Jensen,Daniel Alexander Ackermann,Alexander Jordan,Valdemar Rømer,Søren Sperling,Elisabeth Bendstrup,Casper Falster,Christian B Laursen,Jørn Carlsen,Jens-Ulrik Stæhr Jensen","doi":"10.1183/13993003.00169-2025","DOIUrl":"https://doi.org/10.1183/13993003.00169-2025","url":null,"abstract":"BACKGROUNDElevated pulmonary pressures can lead to right ventricular (RV) dysfunction, worsen respiratory status, and increase overall morbidity in chronic obstructive pulmonary disease (COPD) patients. Yet, little is known about the impact of right sided pressure changes during COPD exacerbations (AECOPD) on patient outcomes.AIMTo determine whether pulmonary pressures are elevated during AECOPD compared to stable phase and to investigate the association between tricuspid regurgitation (TR) gradient during AECOPD and days alive and out of hospital (DAOH).METHODSThis multicenter, prospective study of pulmonary pressures changes in patients with AECOPD and stable phase. Inclusion criteria were diagnosis of COPD and admission with AECOPD. Transthoracic echocardiography (TTE), including TR gradient, tricuspid annular plane systolic excursion (TAPSE), RV diameter, and right atrial parameters, was performed during AECOPD and stable phase.RESULTSOf 250 patients, 232 underwent TTE during AECOPD, and 107 completed stable-phase follow-up. Reasons for incomplete follow-up included death (46), withdrawal (23), poor TTE quality (21), and unmeasurable TR gradients (35). TR gradient increased significantly during AECOPD, with a mean difference of 6.0 mmHg (95% CI: 2.5-9.6), while TAPSE, RV diameter, and right atrial size showed no significant changes. Higher TR gradients during AECOPD correlated with lower DAOH.CONCLUSIONTR gradient were significantly elevated during AECOPD, suggesting that transient right-sided pressure spikes are associated with COPD exacerbations. However, the direction of this association remains unclear, and further research is needed to determine whether right-sided pressure changes contribute to exacerbations or whether exacerbations themselves drive these pressure spikes.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"4 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796894","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":"Pericardial Effusions And Sotatercept Therapy in Pulmonary Arterial Hypertension: A Multicenter Real-World Experience.","authors":"Sandeep Sahay,Nelson Villasmil Hernandez,Yassmin Al Aaraj,Rahul G Argula,Roberto J Bernardo,Meagan Chavarria,Chukwukadibia Ibecheozor,James C Coons,Robyn T Domsic,Terry Fortin,Amy Goodrich-Harris,Gavin Hickey,Truc Ho,Jennifer Kliner,Roberto F Machado,Sudarshan Rajagopal,Michael G Risbano,Denise G Sese,Thenappan Thenappan,Stephen Y Chan","doi":"10.1183/13993003.01040-2025","DOIUrl":"https://doi.org/10.1183/13993003.01040-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"27 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796800","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":"Update of the International Multidisciplinary Classification of the Interstitial Pneumonias: An ERS/ATS Statement.","authors":"Christopher J Ryerson,Ayodeji Adegunsoye,Sara Piciucchi,Lida P Hariri,Yet H Khor,Marlies S Wijsenbeek,Athol U Wells,Amita Sharma,Wendy A Cooper,Katerina Antoniou,Raphael Borie,Aurelie Fabre,Yoshikazu Inoue,Kerri Johannson,Takeshi Johkoh,Leticia Kawana-Dourado,Ella Kazerooni,Toby M Maher,Philip L Molyneaux,Raymond Protti,Claudia Ravaglia,Elisabetta A Renzoni,Ryoko Saito-Koyama,Nicola Sverzellati,Simon L F Walsh,Paul Wolters,Soo-Ryum Yang,William Travis,Andrew Nicholson","doi":"10.1183/13993003.00158-2025","DOIUrl":"https://doi.org/10.1183/13993003.00158-2025","url":null,"abstract":"BACKGROUNDThe 2013 American Thoracic Society/European Respiratory Society Statement on the classification of the idiopathic interstitial pneumonias described 6 major and 2 rare subtypes of idiopathic interstitial pneumonia, as well as recognising unclassifiable disease.OBJECTIVEThe objective of this statement is to update the 2013 classification of interstitial pneumonia.METHODSFive co-chairs identified a committee of 32 experts in the field as well as two individuals with lived experience. Creation of the document was supported by a series of video meetings, first including the full committee and then subgroups assigned to draft specific sections of the document. The classification scheme was developed by consensus.RESULTSThe multidisciplinary committee of experts identified four major advances to the classification of interstitial pneumonia: (1) expansion beyond idiopathic interstitial pneumonias to also include secondary causes; (2) identification of new subcategories and updated terms, including addition of bronchiolocentric interstitial pneumonia as a major pattern as well as changing from acute interstitial pneumonia to idiopathic diffuse alveolar damage and desquamative interstitial pneumonia to alveolar macrophage pneumonia; (3) subclassification of interstitial and alveolar filling disorders, with interstitial disorders further subclassified as fibrotic versus non-fibrotic; and (4) consideration of diagnostic confidence in patient evaluation and management. The committee also provided a comprehensive update on the status of potential molecular tools and identified future research priorities.CONCLUSIONSThis update builds upon the previous classification approach by describing major advances in the classification of interstitial pneumonia over the last decade.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"16 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796899","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":"Episcleral vascular abnormalities in pulmonary arterial hypertension: clinical significance and genetic implications.","authors":"Qin-Hua Zhao,Rui Zhang,Xin-Rong Zhou,Hong-Ling Qiu,Ci-Jun Luo,Hui-Ting Li,Wen-Hui Wu,Ping Yuan,Jing He,Jian Xu,Jin-Ming Liu,Su-Gang Gong,Jing-Fa Zhang,Lan Wang","doi":"10.1183/13993003.00770-2025","DOIUrl":"https://doi.org/10.1183/13993003.00770-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"27 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796900","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":"Butyrate-producing gut bacterium Faecalibacterium prausnitzii protects against bacterial pneumonia.","authors":"Robert F J Kullberg,Christine C A van Linge,Alex F de Vos,Xanthe Brands,Thi Phuong Nam Bui,Joe M Butler,Daniël R Faber,Aswin Verhoeven,René M van den Wijngaard,Wouter J de Jonge,Max Nieuwdorp,Tom van der Poll,Bastiaan W Haak,W Joost Wiersinga","doi":"10.1183/13993003.02208-2024","DOIUrl":"https://doi.org/10.1183/13993003.02208-2024","url":null,"abstract":"BACKGROUNDGut microbiota play a protective role against pneumonia in mice, probably by producing the immunomodulatory short-chain fatty acid butyrate. Yet, butyrate has limited potential for clinical use due to its challenging handling in practice. We performed mouse experiments and translational analyses to determine whether butyrate-producing gut commensals, Faecalibacterium prausnitzii and Anaerobutyricum soehngenii, could provide protection against bacterial pneumonia and serve as next-generation probiotic.METHODSWe pretreated C57BL/6J mice with butyrate, F. prausnitzii or A. soehngenii, and subsequently infected them intranasally with Klebsiella pneumoniae. To assess the relevance in humans, we assessed associations between rectal levels of Faecalibacterium, immune responses, and clinical outcomes in 115 patients with community-acquired pneumonia (CAP), and in a separate validation cohort.RESULTSPretreatment with F. prausnitzii, but not A. soehngenii, protected mice against bacterial pneumonia, as reflected by reduced bacterial growth and dissemination, lessened organ damage and dampened inflammation. Similar to butyrate pretreatment, F. prausnitzii resulted in reduced pulmonary IL-6 and CXCL-1. In humans, gut Faecalibacterium was decreased during CAP compared to matched controls. CAP patients with higher gut Faecalibacterium levels had lower IL-6-producing capacity and downregulated inflammatory gene expression. Higher intestinal Faecalibacterium levels were associated with better clinical outcomes in independent cohorts of CAP and critically ill patients, which remained significant when controlled for potential confounders.CONCLUSIONThis is the first study showing that the gut commensal Faecalibacterium prausnitzii provides protection against bacterial pneumonia and has translational potential. This motivates future studies investigating the clinical potential of F. prausnitzii as novel probiotic for pneumonia.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"68 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796946","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":"Lateral wall collapse from sleep endoscopy and airflow shape predicts hypoglossal nerve stimulation efficacy in obstructive sleep apnea.","authors":"Daniel Vena,Sara Op de Beeck,Hyungchae Yang,Jeffrey Sumner,Dwayne Mann,Tsai-Yu Wang,Atqiya Aishah,Ali Azarbarzin,Ludovico Messineo,Nicole Calianese,Raichel Alex,Neda Esmaeili,Olivier M Vanderveken,David P White,Andrew Wellman,Phillip Huyett,Scott A Sands","doi":"10.1183/13993003.00236-2025","DOIUrl":"https://doi.org/10.1183/13993003.00236-2025","url":null,"abstract":"RATIONALEPatient selection for hypoglossal nerve stimulation (HGNS) for obstructive sleep apnea (OSA) requires assessment of pharyngeal site of collapse using drug-induced sleep endoscopy (DISE).OBJECTIVESThe current study aims to address two key knowledge gaps: First, we prospectively confirm that, among HGNS candidates, reduced HGNS efficacy is associated with oropharyngeal lateral wall (OLW) collapse (Aim 1). Second, given DISE is resource-intensive procedure and delays treatment, we evaluate whether a recently-developed non-invasive method for identifying OLW collapse using airflow shapes is associated with reduced HGNS efficacy (Aim 2).METHODSPatients who underwent DISE, HGNS implantation, and follow-up sleep testing were included in Aim 1 (n=369) as part of an observational cohort study. For Aim 2, airflow data estimating OLW collapse probability were collected during DISE via pneumotachograph (n=138, DISE Flow cohort); and from a home sleep test via nasal cannula for validation (n=46, HST cohort). Linear regression quantified associations between HGNS efficacy (%reduction in AHI) and DISE-determined OLW collapse (Aim 1) or flow-shape-determined OLW collapse (probability score per 2SD; Aim 2), adjusting for baseline AHI.RESULTSCompared to non-OLW collapse, DISE-determined OLW collapse reduced HGNS efficacy [95%CI] by ‒18.0[‒31.9,‒6.2]%. Increased flow-shape-determined OLW collapse probability (Δ2SD) was associated with reduced HGNS efficacy in both DISE Flow (‒24.8 [‒40.4, ‒11.7]%) and HST (‒22.7 [‒50.0, ‒2.6]%) cohorts.CONCLUSIONThis study prospectively validates OLW collapse as a key factor in HGNS failure and shows that airflow-based identification of OLW collapse can effectively estimate HGNS efficacy, representing a significant advancement in patient selection for HGNS.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"22 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796799","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":"Pericardial effusion in sotatercept phase 3 trials: insights from STELLAR and ZENITH.","authors":"Marius M Hoeper,Ioana R Preston,Mardi Gomberg-Maitland,Aaron B Waxman,Yaru Shi,Jianxin Lin,Harald S Mackenzie,Maria José Loureiro,Vallerie V McLaughlin,Marc Humbert, ","doi":"10.1183/13993003.00768-2025","DOIUrl":"https://doi.org/10.1183/13993003.00768-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"122 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796895","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 Sarcoidosis Clinical Trial Endpoints: A Delphi Study.","authors":"Robert P Baughman,Jan C Grutters,Elyse E Lower,Ying Zhou,Marlies Wijsenbeek,Athol U Wells,Marcel Veltkamp,Dominique Valeyre,Paolo Spagnolo,Michelle Sharp,Jacobo Sellares,Ogugua N Obi,Hilario Nunes,Marc A Judson,Daniel A Culver,Francesco Bonella,Arata Azuma,Ingrid H E Korenromp","doi":"10.1183/13993003.00943-2025","DOIUrl":"https://doi.org/10.1183/13993003.00943-2025","url":null,"abstract":"BACKGROUNDCorticosteroids (CS), the most commonly prescribed treatment for sarcoidosis, are associated with significant toxicity, especially with long term usage. New intervention trials designed to reduce or eliminate CS require pertinent and precise clinical trial endpoints. However, consensus is lacking. The aim of the current study is to develop consensus for trial endpoints in pulmonary sarcoidosis.METHODSA Delphi survey was developed by an Expert Panel of 30 sarcoidosis investigators. For Round 1, the panel created 97 statements regarding potential endpoints for a clinical trial of symptomatic pulmonary CS treated sarcoidosis patients. After anonymous voting in Round 2, the 97 statements were refined by all Stakeholders, including the Expert Panel and an additional 70 individuals interested in sarcoidosis therapies.RESULTSAfter Round 2, the Expert Panel achieved consensus for combination endpoints and 38 statements across six domains that supported the following endpoints: prednisone reduction by 50% or discontinuation, 10% predicted or greater improvement in FVC%, FEV1%, and DLCO% predicted, and improvement in the Kings Sarcoidosis Questionnaire (KSQ)-Lung and KSQ-General Health. Additionally, the majority of Stakeholders agreed to all statements which reached consensus by the Expert Panel.CONCLUSIONSUtilizing the Delphi technique, international sarcoidosis experts successfully achieved consensus for 38 specific clinical trial endpoints which can be utilized in the development of novel steroid-sparing and eliminating therapies for pulmonary sarcoidosis.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"103 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144796798","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":"Prognostication in patients with idiopathic pulmonary fibrosis using quantitative airway analysis from HRCT: a retrospective study.","authors":"Yang Nan,Felder N Federico,Stephen Humphries,John A Mackintosh,Christopher Grainge,Helen E Jo,Nicole Goh,Paul N Reynolds,Peter M A Hopkins,Vidya Navaratnam,Yuben Moodley,Haydn Walters,Samantha Ellis,Gregory Keir,Chris Zappala,Tamera Corte,Ian Glaspole,Athol U Wells,Guang Yang,Simon Lf Walsh","doi":"10.1183/13993003.00981-2025","DOIUrl":"https://doi.org/10.1183/13993003.00981-2025","url":null,"abstract":"BACKGROUNDPredicting shorter life expectancy is crucial for prioritizing antifibrotic therapy in fibrotic lung diseases, where progression varies widely, from stability to rapid deterioration. This heterogeneity complicates treatment decisions, emphasizing the need for reliable baseline measures. This study focuses on leveraging artificial intelligence model to address heterogeneity in disease outcomes, focusing on mortality as the ultimate measure of disease trajectory.METHODSThis retrospective study included 1744 anonymised patients who underwent high-resolution CT scanning. The AI model, SABRE (Smart Airway Biomarker Recognition Engine), was developed using data from patients with various lung diseases (n=460, including lung cancer, pneumonia, emphysema, and fibrosis). Then, 1284 high-resolution CT scans with evidence of diffuse FLD from the Australian IPF Registry and OSIC were used for clinical analyses. Airway branches were categorized and quantified by anatomic structures and volumes, followed by multivariable analysis to explore the associations between these categories and patients' progression and mortality, adjusting for disease severity or traditional measurements.RESULTSCox regression identified SABRE-based variables as independent predictors of mortality and progression, even adjusting for disease severity (fibrosis extent, traction bronchiectasis extent, and ILD extent), traditional measures (FVC%, DLCO%, and CPI), and previously reported deep learning algorithms for fibrosis quantification and morphological analysis. Combining SABRE with DLCO significantly improved prognosis utility, yielding an AUC of 0.852 at the first year and a C-index of 0.752.CONCLUSIONSSABRE-based variables capture prognostic signals beyond that provided by traditional measurements, disease severity scores, and established AI-based methods, reflecting the progressiveness and pathogenesis of the disease.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"52 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-07-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144756085","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}