European Respiratory Journal最新文献

{"title":"Decoding the complexity: mechanistic insights into comorbidities in idiopathic pulmonary fibrosis.","authors":"Moisés Selman, Ivette Buendia-Roldan, Annie Pardo","doi":"10.1183/13993003.02418-2024","DOIUrl":"10.1183/13993003.02418-2024","url":null,"abstract":"<p><p>The complex pathogenic relationships between idiopathic pulmonary fibrosis (IPF) and its usually associated comorbidities remain poorly understood. While evidence suggests that some comorbidities may directly influence the development or progression of IPF, or <i>vice versa</i>, whether these associations are causal or arise independently due to shared risk factors, such as ageing, smoking, lifestyle and genetic susceptibility, is still uncertain. Some comorbidities, such as metabolic syndromes, gastro-oesophageal reflux disease and obstructive sleep apnoea, precede the development of IPF. In contrast, others, such as pulmonary hypertension and lung cancer, often become apparent after IPF onset or during its progression. These timing patterns suggest a directional relationship in their associations. The issue is further complicated by the fact that patients often have multiple comorbidities, which may interact and exacerbate one another, creating a vicious cycle. To clarify these correlations, some studies have used causal inference methods (<i>e.g.</i> Mendelian randomisation) and exploration of underlying mechanisms; however, these efforts have not yet generated conclusive insights. In this review, we provide a general overview of the relationship between IPF and its comorbidities, emphasising the pathogenic mechanisms underlying each comorbidity, potential shared pathobiology with IPF and, when available, causal insights from Mendelian randomisation studies.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12095908/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143779508","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":"Clean air prescriptions: investing in healthy lungs and a healthier future.","authors":"José Luis Castro,Maria Neira,Sarah Rylance,Marit Viktoria Pettersen,Samantha Pegoraro","doi":"10.1183/13993003.00186-2025","DOIUrl":"https://doi.org/10.1183/13993003.00186-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"18 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122056","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":"Effectiveness and safety of tofacitinib <i>versus</i> calcineurin inhibitor in interstitial lung disease secondary to anti-MDA5-positive dermatomyositis: a multicentre cohort study.","authors":"Wanlong Wu, Bingpeng Guo, Wenjia Sun, Dan Chen, Wenwen Xu, Zhiwei Chen, Yakai Fu, Yan Ye, Xia Lyu, Zhixin Xue, Kaiwen Wang, Jiangfeng Zhao, Cuiying Xie, Yi Chen, Chunhua Ye, Min Dai, Wei Fan, Jia Li, Xiaodong Wang, Yu Xue, Weiguo Wan, Li Sun, Huaxiang Wu, Qun Luo, Qian Han, Qiong Fu, Shuang Ye","doi":"10.1183/13993003.01488-2024","DOIUrl":"10.1183/13993003.01488-2024","url":null,"abstract":"<p><strong>Objective: </strong>To compare the effectiveness and safety of tofacitinib <i>versus</i> calcineurin inhibitor (CNI) as initial immunosuppressive regimen for anti-melanoma differentiation-associated gene 5-positive dermatomyositis with interstitial lung disease (MDA5⁺DM-ILD).</p><p><strong>Methods: </strong>Adult Chinese patients with newly diagnosed MDA5⁺DM-ILD (ILD course <3 months) from five tertiary referral centres between April 2014 and January 2023 were included in this retrospective cohort study. The primary effectiveness end-point was lung transplantation-free survival within 1 year. Propensity score-based inverse probability of treatment weighting (IPTW) was applied for adjustment in this real-world study.</p><p><strong>Results: </strong>In the eligible cohort, a total of 94 (32.4%) and 105 (46.7%) patients died or underwent lung transplantation within 1 year in the tofacitinib group (n=290) and the CNI group (n=225), respectively. After adjustment by IPTW, patients' lung transplantation-free survival rate within 1 year was significantly higher in the tofacitinib group compared to the CNI group (log-rank p=0.013). Multivariable Cox analysis performed in the IPTW dataset revealed that the hazard ratio of tofacitinib <i>versus</i> CNI for 1-year survival was 0.72 (95% CI 0.56-0.94; p=0.013). The adjusted difference of survival rate was 9.3% (95% CI 2.8-15.8%). Alternative analytic strategies yielded consistent results in sensitivity analyses. Patients aged <60 years, without rapidly progressive ILD, or with baseline arterial oxygen tension/inspiratory oxygen fraction ≥300 mmHg might benefit more from tofacitinib. Opportunistic infection was the major treatment-related serious adverse event, with generally comparable incidence (42.4% <i>versus</i> 45.3%).</p><p><strong>Conclusion: </strong>In this large multicentre cohort study, tofacitinib showed significantly more benefits for 1-year lung transplantation-free survival than calcineurin inhibitors in MDA5⁺DM-ILD.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12095906/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143064846","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":"Rehabilitation for persons with long COVID beyond the recovery phase of SARS-CoV-2 infection.","authors":"Milo A Puhan,Kaba Dalla Lana","doi":"10.1183/13993003.00239-2025","DOIUrl":"https://doi.org/10.1183/13993003.00239-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"33 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122054","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":"Equity in research: a global consensus statement on the urgency of including children in long COVID clinical trials.","authors":"Lael M Yonker,Binita Kane,Etheresia Pretorius,David Putrino,Sammie McFarland,Petter Brodin,Kanecia O Zimmerman,Daniel Munblit,Peter C Rowe,Theo Vos,David Warburton,Terence Stephenson,Danilo Buonsenso,","doi":"10.1183/13993003.00092-2025","DOIUrl":"https://doi.org/10.1183/13993003.00092-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"137 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122055","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":"Tofacitinib in anti-MDA5-positive dermatomyositis-associated interstitial lung disease: a new standard of care emerges.","authors":"Toyoshi Yanagihara,Reza D Mirza,Martin R J Kolb","doi":"10.1183/13993003.00458-2025","DOIUrl":"https://doi.org/10.1183/13993003.00458-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"20 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122057","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":"Phenotyping the responses to systemic corticosteroids in the management of asthma attacks (PRISMA).","authors":"Carlos Celis-Preciado, Simon Leclerc, Martine Duval, Dominic O Cliche, Lucie Brazeau, Félix-Antoine Vézina, Marylène Dussault, Pierre Larivée, Samuel Lemaire-Paquette, Simon Lévesque, Philippe Lachapelle, Simon Couillard","doi":"10.1183/13993003.02391-2024","DOIUrl":"10.1183/13993003.02391-2024","url":null,"abstract":"<p><strong>Background: </strong>Asthma attacks are heterogeneous. It is not known whether the response to oral corticosteroids (OCS) in acute asthma varies according to type 2 (T2) inflammatory biomarkers, blood eosinophil count (BEC) and fractional exhaled nitric oxide (<i>F</i> _ENO). We aimed to explore the relationship between T2 biomarkers and response to OCS in acute asthma.</p><p><strong>Methods: </strong>We conducted a longitudinal observational study of people experiencing an asthma attack evaluated before and after a 7-day OCS course. The primary outcome was post-bronchodilator change in forced expiratory volume in 1 s (FEV₁) according to ordinal BEC-<i>F</i> _ENO three-group categories (T2-Low/Low: BEC <0.15×10⁹ cells·L^-1 and <i>F</i> _ENO <25 ppb; T2-High/High: BEC ≥0.30×10⁹ cells·L^-1 and <i>F</i> _ENO ≥35 ppb; T2-Mid: not meeting Low/Low or High/High criteria). A key secondary outcome was the change in Asthma Control Questionnaire-5 score. Exploratory outcomes included OCS-attributable adverse events.</p><p><strong>Results: </strong>53 people were enrolled, with 16 (30%) T2-Low/Low, 27 (51%) T2-Mid and 10 (19%) T2-High/High asthma attacks. Post-bronchodilator FEV₁ changes increased with combined BEC-<i>F</i> _ENO elevation (p for interaction=0.007), peaking in the T2-High/High phenotype (0.390±0.512 L, p for trend<0.0001). Conversely, T2-Low/Low attacks showed nonsignificant FEV₁ changes (0.017±0.153 L). In univariable and multivariable analyses, only ordinal BEC-<i>F</i> _ENO stratification, not symptoms nor FEV₁, predicted subsequent post-bronchodilator FEV₁ improvement. All patients had improved Asthma Control Questionnaire-5 score, numerically peaking in the T2-High/High phenotype (-1.58±0.60, p for trend=0.08). All groups experienced similar OCS-attributable adverse events, with 33 patients (62%) reporting at least one event.</p><p><strong>Conclusions: </strong>We found that objective improvement following OCS is confined to T2-High events. As in chronic asthma, greater T2 burden identifies a distinct clinical and therapeutic trajectory, whereas OCS‑related adverse events are uniformly distributed.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12095907/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413866","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":"Sputum metagenomics in bronchiectasis reveals pan-European variation: an EMBARC-BRIDGE study.","authors":"Kai Xian Thng,Pei Yee Tiew,Micheál Mac Aogáin,Jayanth Kumar Narayana,Tavleen Kaur Jaggi,Fransiskus Xaverius Ivan,Morven Shuttleworth,Merete B Long,Hollian Richardson,Holly Lind,Daniela Alferes de Lima Headley,Kara Robertson,Jennifer Pollock,Pieter C Goeminne,Michal Shteinberg,Anthony De Soyza,Stefano Aliberti,Josje Altenburg,Charles S Haworth,Oriol Sibila,Eva Polverino,Michael R Loebinger,Felix C Ringshausen,Natalie Lorent,Katerina Dimakou,Amelia Shoemark,James D Chalmers,Sanjay H Chotirmall","doi":"10.1183/13993003.00054-2025","DOIUrl":"https://doi.org/10.1183/13993003.00054-2025","url":null,"abstract":"BACKGROUNDThe EMBARC registry shows considerable variation in culturable microbes in sputum between different European countries. The additive role of next generation metagenomic sequencing remains unexplored and association with antimicrobial resistomes unknown.METHODSWe prospectively assessed sputum from N=349 individuals recruited into the EMBARC-BRIDGE study with next-generation shotgun metagenomic sequencing including three European regions: Northern and Western Europe, Southern Europe and the United Kingdom, including samples from ten European countries. Microbiome and resistome profiles were assessed in relation to clinical outcomes.RESULTSNext generation metagenomic sequencing reproduced differences between countries in microbial profiles previously shown by culture in the EMBARC study. Metagenomics provided enhanced detection for some bronchiectasis pathogens including P. aeruginosa, H. influenzae and S. pneumoniae. Three metagenomic microbial clusters dominated by the genera Pseudomonas, Streptococcus and Haemophilus demonstrated pan-European but variable distribution. Diverse resistomes, linked to underlying microbiomes, were identified across Europe, with significantly higher diversity of resistance gene determinants in Southern Europe. Resistome composition significantly differed between regions characterised by regionally contrasting multi-drug-resistant profiles. The EMBARC-BRIDGE cohort validated established bronchiectasis resistotypes: RT1 and RT2, which occur at varying frequency across regions. Despite geographic variation in microbiome and resistome profiles in bronchiectasis across Europe, analogous antimicrobial resistance gene profiles associate with the key bronchiectasis genera Pseudomonas, Streptococcus and Haemophilus, independent of country or region.CONCLUSIONSputum metagenomics confirms and extends prior observations of regional variation in bronchiectasis microbiology. Important variation in the distribution of pathogens and antimicrobial resistance genes has implications for antimicrobial practices across Europe.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"60 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122050","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":"Comparability, acceptability and longitudinal adherence with digital emPHasis-10 in pulmonary arterial hypertension.","authors":"Joseph Newman,Frances Varian,Felicity Hitchcock,Rebecca Burney,Gregg Harry Rawlings,John Harrington,Ze Ming Goh,Jenna Ablott,David G Kiely,Iain Armstrong,A A Roger Thompson,Jill Carlton,Elin Haf Davies,Alexander Rothman,Mark Toshner","doi":"10.1183/13993003.00198-2025","DOIUrl":"https://doi.org/10.1183/13993003.00198-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"2 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122051","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":"Impaired IgA Mucosal Immunity Following Lung Transplantation: A Potential Trigger for Bronchiolitis Obliterans Syndrome.","authors":"François M Carlier,Bruno Detry,Jérôme Ambroise,Nicolas Heddebaut,Thomas Planté-Bordeneuve,Aurélie Daumerie,Elisabeth Longchampt,Loïc Falque,Martine Reynaud-Gaubert,Sandrine Hirschi,Jean-François Mornex,Xavier Demant,Adrien Tissot,Jérôme Le Pavec,Vincent Bunel,David Lair,Marina Pretolani,Alexandre Vallée,Charles Pilette,Olivier Brugière,","doi":"10.1183/13993003.02212-2024","DOIUrl":"https://doi.org/10.1183/13993003.02212-2024","url":null,"abstract":"RATIONALEBronchiolitis obliterans syndrome (BOS) limits long-term survival after lung transplantation (LuTx) and may be triggered by infections. As immunoglobulin (Ig)A is crucial to ensure adequate mucosal immunity, we explored whether IgA-related mucosal immunity is impaired in BOS.METHODSSixty LuTx recipients from the COLT cohort were retrospectively included. All participants were in stable condition within the first year post-transplant. At 3.5 years post-LuTx, 30 remained stable and 30 had developed BOS. Bronchoalveolar lavage fluid (BALF) and sera collected pre-transplant and at 6 (M6) and 12 months (M12) post-transplant were assessed for monomeric IgA, secretory (S)-IgA, secretory component (SC) and cytokine profiling. Second, bronchiolar polymeric Ig receptor (pIgR) expression and subepithelial IgA-producing B-cell numbers were compared across graft tissue samples from 54 LuTx recipients classified as stable, pre-BOS, BOS or end-stage BOS.RESULTSS-IgA levels in BALF decreased between M6 and M12 (p=0.0001) and were reduced in BOS patients at M12 (p=0.0018). Patients with lower S-IgA levels had higher infection rates. BOS patients exhibited elevated SC levels in serum (p<0.01). Both reduced S-IgA in BALF and increased SC in serum were associated with higher risk of BOS. Lastly, a reduction in bronchiolar pIgR expression was observed in BOS patients (p=0.0001), that paralleled BOS severity.CONCLUSIONSThis study demonstrates an early impairment of mucosal IgA immunity in LuTx patients, which was linked to the later development of BOS, suggesting that IgA-related markers may serve as early predictors of BOS onset.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"31 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-05-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144122128","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}