European Respiratory Journal最新文献

{"title":"Withdrawal of prostacyclin pathway therapies after initiation of sotatercept treatment in patients with pulmonary arterial hypertension.","authors":"Karen M Olsson,Jan Fuge,Da-Hee Park,Jan C Kamp,Marius M Hoeper","doi":"10.1183/13993003.00064-2025","DOIUrl":"https://doi.org/10.1183/13993003.00064-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"13 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143872060","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 eosinophil counts, airway infections and inhaled antibiotic treatment in bronchiectasis.","authors":"Ming-Xin Shi,Shu-Jun Guo,Jun-Qing Yue,Xiao-Xian Zhang,Zhen-Feng He,Hui-Min Li,Xiao-Fen Zhang,Zhao-Ming Chen,Lai-Jian Cen,Cui-Xia Pan,Rui-di Tang,Sheng-Zhu Lin,Jia-Hui He,Dan-Yuan Xu,Zi-Wei Chen,Meng-Zhang He,Yong-Hua Gao,Wei-Jie Guan","doi":"10.1183/13993003.00518-2025","DOIUrl":"https://doi.org/10.1183/13993003.00518-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"53 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143872058","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":"Real-world monitoring of elexacaftor-tezacaftor-ivacaftor trough concentrations in adults with cystic fibrosis.","authors":"Paulette Magnas,Sihem Benaboud,Jennifer Da Silva,Clémence Martin,Reem Kanaan,Nicolas Carlier,Isabelle Honoré,Johanna Fesenbeckh,Naim Bouazza,Franz Foissac,Jean-Marc Treluyer,Pierre-Régis Burgel","doi":"10.1183/13993003.02490-2024","DOIUrl":"https://doi.org/10.1183/13993003.02490-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"13 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871995","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":"How to diagnose TB in migrants? A systematic review of reviews and decision tree analytical modelling exercise to evaluate properties for single and combined TB screening tests.","authors":"Dominik Zenner,Hassan Haghparast-Bidgoli,Tahreem Chaudhry,Ibrahim Abubakar,Frank Cobelens","doi":"10.1183/13993003.02000-2024","DOIUrl":"https://doi.org/10.1183/13993003.02000-2024","url":null,"abstract":"BACKGROUNDOptimising Tuberculosis (TB) disease testing algorithms is fundamental to ensure the effectiveness and cost-effectiveness of migrant screening programmes, including better understanding of the individual and combined screening test properties. The aim of our study was to estimate pooled TB test properties from the literature and combining them in decision analytical modelling with a focus on whether tests used for the diagnosis of TB infection might add value to these algorithms.METHODSWe performed a systematic review of reviews (RoR) of diagnostic tests for active TB, searching PubMed, Embase, Web of Science and Cochrane library and pooled test properties extracted from original papers included in reviews. We used these pooled results in a decision tree analysis to estimate test properties for common migrant screening algorithms.FINDINGSWe retrieved 1477 records and included 32 reviews, including data from 437 original studies for 18 TB tests, providing pooled results for 13. Our modelling showed that algorithms with interferon gamma release assays (IGRAs) had the highest diagnostic odds ratios (e.g. QuantiFERON/Chest X-Ray (CXR, TB abnormalities)/Xpert dOR 24 670; 95% confidence intervals 11 630-52 328) and high positive predictive values. Best sensitivities were achieved for combinations with parallel cough/CXR screening followed by Xpert (0.88; CI 0.86-0.90) or Ultra (0.92; 0.90-0.94) as well as T-Spot.TB followed by parallel symptom/CXR screening and Ultra (0.81; 0.78-0.83) or Xpert (0.77; 0.75-0.80).INTERPRETATIONThe significant test accuracy benefit of adding IGRAs to an active TB screening pathway will help inform clinicians and policy makers deciding on the most effective screening algorithms.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"17 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871987","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":"Idiopathic pulmonary fibrosis and murine models of pulmonary fibrosis: Correlation of decline in lung function.","authors":"Ashley Chang,Jhon Tolentino Sia,Garrett E Casper,Seth R Wood,Lance Markham,Luke L Westhoff,Aubrianna J Saxton,Gregory L Snow,Pam M Van Ry,Ganesh Raghu","doi":"10.1183/13993003.02317-2024","DOIUrl":"https://doi.org/10.1183/13993003.02317-2024","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"7 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143871994","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":"Pleural fluid proteomics from patients with pleural infection shows signatures of diverse neutrophilic responses: The Oxford Pleural Infection Endotyping Study (TORPIDS-2).","authors":"Nikolaos I Kanellakis,Elie Antoun,Kiki Cano-Gamez,Julia Chu,Nikita Manoharan,Georgina Berridge,Iolanda Vendrell,Zheqing Zhang,John P Corcoran,Alguili Elsheikh,Tao Dong,Roman Fischer,Justin P Whalley,Julian C Knight,Najib M Rahman","doi":"10.1183/13993003.00010-2025","DOIUrl":"https://doi.org/10.1183/13993003.00010-2025","url":null,"abstract":"BACKGROUNDPleural infection is a complex disease with poor clinical outcomes and increasing incidence worldwide, yet its biological endotypes remain unknown.METHODSWe analysed 80 pleural fluid samples from the PILOT study, a prospective study on pleural infection, using unlabelled mass spectrometry. A total of 449 proteins were retained after filtering. Unsupervised hierarchical clustering and UMAP analyses were used to cluster samples and pathway analysis was performed to identify the biological processes. Protein signatures as identified by the pathway analysis were compared to microbiology as defined by 16S rRNA next generation sequencing. Spearman and exact Fischer's methods were used for correlation assessment.RESULTSHigher neutrophil degranulation was correlated with increased glycolysis (OR=281, p<2.2E-16) and pentose phosphate activation (OR=371.45, p<2.2E-16). Samples dominated by Streptococcus pneumoniae exhibited higher neutrophil degranulation (OR=12.08, p=0.005), glycolysis (OR=11.4, p=0.006), and pentose phosphate activity (OR=12.82, p=0.004). On the other hand, samples dominated by anaerobes and Gram-negative bacteria exhibited lower neutrophil degranulation (OR=0.15, p=0.01, glycolysis (OR=0.14, p=0.01), and pentose phosphate activity (OR=0.07, p=0.001). Increased activity of the liver and retinoid X receptors (LXR-RXR) pathway was associated with lower risk of one-year mortality (OR=0.24, p=0.04).CONCLUSIONSThese findings suggest that pleural infection patients exhibit diverse responses of neutrophil mediated immunity, glycolysis, and pentose phosphate activation which are associated with microbiology. Therapeutic targeting of the LXR-RXR pathway with agonists is a possible treatment approach.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"68 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143849375","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":"Prognostic value of cardiopulmonary exercise testing in pulmonary arterial hypertension.","authors":"Andrea Baccelli,Rocco F Rinaldo,Gulammehdi Haji,Rachel J Davies,Francesco Lo Giudice,Wendy Gin-Sing,Beatrice Vigo,Stefano Centanni,J Simon R Gibbs,Luke S Howard","doi":"10.1183/13993003.02026-2024","DOIUrl":"https://doi.org/10.1183/13993003.02026-2024","url":null,"abstract":"BACKGROUNDCurrent guidelines recommend a four-strata model based on World Health Organization functional class (WHO-FC), 6-min walk distance (6MWD), and serum levels of brain natriuretic peptide (BNP) or N-terminal pro-BNP (NT-proBNP) for risk stratification in patients with pulmonary arterial hypertension (PAH) during follow-up. We explored the relevance of using cardiopulmonary exercise testing (CPET) as the exercise parameter in place of 6MWD at first reassessment after treatment initiation in PAH.METHODSIncident treatment-naïve patients with idiopathic, heritable, drug/toxins-induced, and connective tissue disease-associated PAH between 2010 and 2022 were analysed. Correlations between CPET and haemodynamic and right ventricular (RV) function parameters were explored and those which were significant were carried forward to assess association with survival. Independent predictors were used to derive a four-strata CPET score.RESULTS262 patients were included. CPET parameters showed better correlations with haemodynamics and RV function than 6MWD. The CPET score included peak oxygen uptake (peak VO2), the slope relating ventilation to carbon dioxide production (VE/VCO2 slope), and peak oxygen pulse. The four-strata model based on WHO-FC, BNP, and CPET score predicted survival at the time of the first re-evaluation, with better accuracy than the model including 6MWD (c-index 0.81 versus 0.71). The CPET score on its own also performed well (c-index 0.82) with a greater spread between categories. Treatment-associated changes in peak VO2 and oxygen pulse predicted survival, while changes in 6MWD did not.CONCLUSIONSA simplified four-strata CPET score either alone or included with BNP and WHO-FC accurately predicts survival at follow-up in PAH.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"60 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143822746","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 in children aged ≥6 years with cystic fibrosis heterozygous for F508del and a minimal function mutation: Results from a 96-week open-label extension study.","authors":"Marcus A Mall,Claire E Wainwright,Julian Legg,Mark Chilvers,Sylvia Gartner,Anna-Maria Dittrich,Florian Stehling,Sarah Conner,Sebastian Grant,Nina Suresh,Tanya G Weinstock,Jane C Davies,","doi":"10.1183/13993003.02435-2024","DOIUrl":"https://doi.org/10.1183/13993003.02435-2024","url":null,"abstract":"AIMSElexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious and safe in children aged 6-11 years with cystic fibrosis (CF) heterozygous for F508del and a minimal function CFTR variant (F/MF genotypes) in a 24-week, placebo-controlled trial. We conducted a 96-week open-label extension study for children who completed the 24-week parent study.METHODSIn this phase 3b extension study, dosing was based on weight and age with children weighing <30 kg and aged <12 years receiving ELX 100 mg once daily (qd), TEZ 50 mg qd, and IVA 75 mg every 12 h (q12) and children ≥30 kg or ≥12 years receiving ELX 200 mg qd, TEZ 100 mg qd, and IVA 150 mg q12. Primary endpoint was safety and tolerability. Secondary and other efficacy endpoints included absolute changes from parent study baseline in sweat chloride concentration, LCI2.5, ppFEV1, and CFQ-R respiratory domain score.RESULTSA total of 120 children were enrolled and dosed. One hundred and eighteen children (98.3%) had adverse events (AEs), which for most were mild (43.3%) or moderate (48.3%) in severity. The most common AEs (≥20% of children) were COVID-19 (58.3%), cough (51.7%), nasopharyngitis (45.0%), pyrexia (40.0%), headache (37.5%), upper respiratory tract infection (30.8%), oropharyngeal pain (26.7%), rhinitis (24.2%), abdominal pain (22.5%), and vomiting (20.0%). Children who transitioned from the placebo and ELX/TEZ/IVA groups of the parent study had improvements from parent study baseline at Week 96 in mean sweat chloride concentration (-57.3 [95% CI: -61.6, -52.9] and -57.5 [95% CI: -62.0, -53.0] mmol·L-1), LCI2..5 (-1.74 [95% CI: -2.09, -1.38] and -2.35 [95% CI: -2.72, -1.97] units), ppFEV1 (6.1 [95% CI: 2.6, 9.7] and 6.9 [95% CI: 3.2, 10.5] percentage points), and CFQ-R respiratory domain score (6.6 [95% CI: 2.5, 10.8] and 2.6 [95% CI: -1.6, 6.8] points).CONCLUSIONSELX/TEZ/IVA treatment was generally safe and well-tolerated, with a safety profile consistent with parent study and older age groups. After starting ELX/TEZ/IVA, children had robust improvements in sweat chloride concentration and lung function that were maintained through 96 weeks. These results demonstrate the safety and durable efficacy of ELX/TEZ/IVA in this pediatric population. (Clinical Trials.gov, NCT04545515; EudraCT, 2020-001404-42).","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"75 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143822735","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":"Relationship between theratyping in nasal epithelial cells and clinical outcomes in people with cystic fibrosis.","authors":"Felix Ratjen,Sanja Stanojevic,Tarini Gunawardena,Paul D W Eckford,Julie Avolio,Michelle Shaw,Claire Bartlett,Hong Ouyang,Theo J Moraes,Tanja Gonska,Christine Bear","doi":"10.1183/13993003.01855-2024","DOIUrl":"https://doi.org/10.1183/13993003.01855-2024","url":null,"abstract":"BACKGROUNDIn people with cystic fibrosis (pwCF), human nasal epithelial cultures (HNECs) can be used to assess response to CFTR modulators. However, thresholds of in vitro responses that predict clinical benefit remain poorly understood. In this study we describe the concordance between in vitro response in HNECs and clinical outcomes in pwCF harbouring the F508del variant, treated with either Lumacaftor/Ivacaftor, Tezacaftor/Ivacaftor or Elexacaftor/Tezacaftor/Ivacaftor.METHODSResponse of HNECs to CFTR modulators was assessed by CFTR-mediated chloride current stimulated by forskolin or inhibited by CFTRInh-172 in both pwCF and healthy controls. Clinical response was defined as the change in Forced Expiratory Volume in 1 s (FEV1), Lung Clearance Index (LCI), sweat chloride or the cystic fibrosis questionnaire respiratory domain (CFQr) between baseline and within 3 months after the start of modulator treatment.RESULTSIn 58 unique in vitro:clinical pairs, in vitro measures of functional rescue correlated with changes in FEV1, LCI and sweat chloride, but not CFQr. The concordance between in vitro response and clinical outcomes was highest when a composite outcome was used. For example, an in vitro response of 10% of healthy controls had positive and negative predictive values of 90.5 and 100%, respectively, for a clinical response in either FEV1, LCI or sweat chloride.CONCLUSIONSWe identified thresholds of nasal epithelial cell theratype response in pwCF to predict clinical benefit from CFTR modulator therapy. The utility of this therapy testing platform to predict a clinical response improves when multiple clinical outcome measures are combined.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"5 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2025-04-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143822696","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":"2024 imaging criteria for allergic bronchopulmonary aspergillosis: which diagnostic cut-offs? Are chest radiograph and CT comparable?","authors":"Gael Dournes, Ilyes Benlala, Patrick Berger","doi":"10.1183/13993003.00089-2025","DOIUrl":"https://doi.org/10.1183/13993003.00089-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"65 4","pages":""},"PeriodicalIF":16.6,"publicationDate":"2025-04-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143779512","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}