Thorax最新文献

{"title":"Familial pulmonary fibrosis: a UK consensus framework for the investigation and management of patients and their relatives","authors":"Anne Ramsay Bowden,Deborah J Morris-Rosendahl,Helen Hanson,Stefan Stanel,Michael A Gibbons,Elisabetta Renzoni,Felix Chua,John F Blaikley,Helen Parfrey,Nik Hirani,Pilar Rivera-Ortega,Jennifer A Dickens","doi":"10.1136/thorax-2026-225625","DOIUrl":"https://doi.org/10.1136/thorax-2026-225625","url":null,"abstract":"Background: There is a growing recognition that genetic predisposition contributes to the development of fibrotic interstitial lung disease. Adult onset monogenic disease is most commonly due to dysfunctional telomere maintenance, with a small proportion caused by surfactant biology disorders. These conditions can be associated with additional intrapulmonary and extrapulmonary features which themselves may require surveillance or treatment, making it important to make a genetic diagnosis. The recent introduction in England of a genetic testing panel accessible to respiratory physicians means that genetic information for these individuals is increasingly available but there is currently little standardisation of the subsequent management of patients and their relatives, which can be complex. Aims: This consensus considers the causes and clinical features of familial pulmonary fibrosis and provides a suggested framework for genetic investigation and clinical management of both patients and their relatives. Narrative: We suggest an initial workup that may help identify those with monogenic disease, with focus on key points in the history and examination that may identify features of a telomere or surfactant biology disorder. We highlight that clinical and radiological presentations are diverse and discuss the importance of making a genetic diagnosis to inform multidisciplinary management and facilitate screening of close family members. We also discuss the many outstanding uncertainties and challenges, including the investigation and management of non-monogenic familial pulmonary fibrosis and the management of asymptomatic family members who have inherited a potentially disease-causing genetic variant. Conclusions: We suggest a pathway for the workup and management of patients with familial pulmonary fibrosis, aiming to standardise clinical care for patients and their relatives and provide a framework for the development of a national database to facilitate disease phenotyping and clinical research to improve the evidence base for clinical practice.","PeriodicalId":23284,"journal":{"name":"Thorax","volume":"10 1","pages":"thorax-2026-225625"},"PeriodicalIF":10.0,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148923234","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":"Effects of hyperoxia on pulmonary inflammation and organ injury in a human in vivo model (HIPI): a randomised controlled trial","authors":"Dermot Linden,Delia Dorrian,Michael Glenn,Shikha Tandel,Thea Mawhinney,Karl Welzenbach,Matthew J Rowland,Ming Yang,Laurent Klein,Emanuele Randazzo,Miriam Sindelar,Joerg Schlotterbeck,Alexander Cattini,Ahilanandan Dushianthan,Emma Crossley,Michael McKelvey,Melanie Bailey,John Conlon,Sharon Carr,Judy Bradley,Clifford C Taggart,David A Simpson,Joseph Kidney,Cecilia M O’Kane,Daniel Francis McAuley","doi":"10.1136/thorax-2026-225321","DOIUrl":"https://doi.org/10.1136/thorax-2026-225321","url":null,"abstract":"Introduction It is uncertain whether hyperoxia contributes to adverse intensive care unit outcomes. Potential harm is likely mediated by oxidative stress driving pulmonary and systemic inflammation. Methods We conducted a randomised, double-blind, placebo-controlled trial to test the hypothesis that hyperoxia modifies pulmonary and systemic inflammation following inhaled lipopolysaccharide (LPS) challenge. Healthy adult volunteers were randomised (1:1) to receive hyperoxia administered via high-flow nasal oxygen (FiO 2 1.0, 60 L/min) or synthetic medical air for 6 hours. Bronchoalveolar lavage (BAL) was performed at 6 hours following inhalation of 50 µg of LPS. Blood was collected at baseline, 6 hours and 24 hours. The primary outcome was BAL interleukin-8 (IL-8). Secondary outcome measures included markers of pulmonary and systemic inflammation. Untargeted metabolomic and lipidomic profiling of BAL fluid and plasma was performed by liquid chromatography–mass spectrometry to investigate oxidative stress. Transcriptomic analysis was also performed on whole blood. Results Between January 2023 and March 2024, twenty-two participants were recruited and completed all study procedures. BAL IL-8 did not differ between groups at 6 hours (hyperoxia 375.7 pg/mL (IQR 351.1–467.7) vs placebo 331.9 pg/mL (IQR 251.6–459), p=0.33). No between-group differences were observed in BAL cytokines or markers of systemic inflammation. Hyperoxia was associated with an approximately fourfold increase in BAL reduced glutathione (unadjusted p=0.02). Hyperoxia was associated with distinct transcriptional responses at 24 hours with 175 differentially expressed genes (padj <0.05), including upregulation of genes related to extracellular matrix remodelling, platelet activation and endothelial repair. Discussion In a human inhaled LPS challenge model, 6 hours of hyperoxia did not increase markers of pulmonary or systemic inflammation. Trial registration number NCT05414370 .","PeriodicalId":23284,"journal":{"name":"Thorax","volume":"4 1","pages":"thorax-2026-225321"},"PeriodicalIF":10.0,"publicationDate":"2026-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148923236","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":"'OSA and visceral obesity: a self-perpetuating cycle'.","authors":"Niranjan Ananda Setty, Bhajan Singh","doi":"10.1136/thorax-2026-225557","DOIUrl":"https://doi.org/10.1136/thorax-2026-225557","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148921069","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 the snapshot: childhood immune trajectories and the evolving asthma syndrome.","authors":"Mohammad Leily, Syed Hasan Arshad","doi":"10.1136/thorax-2026-225644","DOIUrl":"https://doi.org/10.1136/thorax-2026-225644","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148921086","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":"Tuberculosis during JAK inhibitor or TNF-α inhibitors therapy: a disproportionality analysis from WHO pharmacovigilance database.","authors":"Thomas Maitre, Kevin Bihan, Nicolas Veziris, Cendrine Godet, Antoine Parrot, Michel Djibre, Benedicte Lebrun Vignes, Jacques Cadranel","doi":"10.1136/thorax-2025-223332","DOIUrl":"https://doi.org/10.1136/thorax-2025-223332","url":null,"abstract":"<p><p>Small-molecule oral Janus kinase (JAK) inhibitors are increasingly used for treatment of digestive and rheumatic inflammatory diseases or haematological disorders. Based on limited data, the risk of tuberculosis (TB) has been reported but considered lower compared with that observed with TNF-α inhibitors a finding we propose to clarify using the WHO VigiBase pharmacovigilance database.We performed a disproportionality analysis of drug-related TB infection using VigiBase. An information component 0.25 (IC0.25) >0 was considered statistically significant. Data were extracted on 09/12/2024. Main characteristics of patients with respiratory infection under JAK and TNF-α inhibitors were analysed.Globally, the mean IC0.25 for TB was 2.0 and 3.2 for JAK and TNFα inhibitors, respectively. Among JAK inhibitors, the risk of TB varied and appeared significant for ruxolitinib, baricitinib, tofacitinib and upadacitinib. A total of 695 patients with TB occurring during JAK inhibitor therapy were included. We observed a higher proportion of reported cases among women (54.7%), aged from 45 to 64 years (35.7%). Extra-thoracic TB involvements were reported in 133 cases (66%). TB cases were most frequently reported among patients receiving tofacitinib (n=330, 47.5%), ruxolitinib (n=217, 31.1%) and upadacitinib (n=102, 14.6%). Overall, 42 patients (5.9%) died with active TB.JAK inhibitors are associated with an increased risk of TB. These results argue to recommend latent TB infection screening and systematic anti-TB preventive therapy even in low endemic countries, before initiating JAK inhibitor therapy.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148921092","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":"Garnet mosaic sign on thoracoscopy in pulmonary angiosarcoma.","authors":"Yuto Yoshida, Norio Kodaka, Takaharu Kiribayashi, Yuki Yokouchi, Hiroto Matsuse","doi":"10.1136/thorax-2026-225566","DOIUrl":"https://doi.org/10.1136/thorax-2026-225566","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-09-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148921077","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":"Acute cardiorespiratory responses to low-load blood-flow restriction vs high-load resistance exercise in COPD: a randomised crossover trial.","authors":"Manuel Kuhn, Christian F Clarenbach, Adrian Kläy, Malcolm Kohler, Diego M Baur, Oliver Senn, Thomas Radtke, Sarah R Haile, Laura C Mayer, Noriane A Sievi, Dario Kohlbrenner","doi":"10.1136/thorax-2026-225047","DOIUrl":"https://doi.org/10.1136/thorax-2026-225047","url":null,"abstract":"<p><strong>Introduction: </strong>Dyspnoea is a major barrier to effective pulmonary rehabilitation in patients with chronic obstructive pulmonary disease (COPD). Low-load blood flow restriction (LL-BFR) resistance exercise may reduce ventilatory demand and dyspnoea burden compared with traditional high-load resistance exercise (HL-TRA). However, its acute physiological and perceptual effects in COPD remain unclear. We compared the acute physiological and perceptual responses to LL-BFR and HL-TRA in patients with COPD.</p><p><strong>Methods: </strong>In a randomised crossover trial, 24 patients with moderate to severe COPD performed LL-BFR (30% of one-repetition maximum (1RM); four sets separated by three rest periods) and HL-TRA (70% of 1RM; three sets separated by two rest periods). Ventilation (V̇E), tidal volume (VT), oxygen uptake (V̇O₂), carbon dioxide output (V̇CO₂), heart rate and ratings of perceived exertion (RPE) were measured throughout. Linear mixed models with participant-level random intercepts were used to compare responses between conditions.</p><p><strong>Results: </strong>LL-BFR resulted in lower V̇E (-2.2 L/min), VT (-0.08 L), V̇O₂ (-0.04 L/min) and V̇CO₂ (-0.07 L/min) compared with HL-TRA. Heart rate and breathing rate were similar across both conditions. Dyspnoea was significantly reduced (RPE -0.7), while leg effort increased (RPE +0.7). LL-BFR was well tolerated, with no adverse events.</p><p><strong>Discussion: </strong>Compared with HL-TRA, LL-BFR was associated with lower ventilatory and metabolic demands and lower perceived dyspnoea despite higher perceived leg exertion. These findings suggest that LL-BFR may represent a useful resistance training strategy for patients with COPD who are primarily limited by dyspnoea during exercise.</p><p><strong>Trial registration number: </strong>NCT05163600.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148832628","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":"Journal club.","authors":"Muhammad Sameed","doi":"10.1136/thorax-2026-225858","DOIUrl":"https://doi.org/10.1136/thorax-2026-225858","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148819756","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":"Mucus plugs in COPD: another reason for not losing weight?","authors":"Jørgen Vestbo","doi":"10.1136/thorax-2026-225548","DOIUrl":"https://doi.org/10.1136/thorax-2026-225548","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-08-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148819750","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 embolism following mycoplasma pneumoniae infection in a child.","authors":"Guodong Ding, Suling Wu, Yutong Gu, Angela Vinturache, Yongjun Zhang","doi":"10.1136/thorax-2026-225225","DOIUrl":"https://doi.org/10.1136/thorax-2026-225225","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":9.1,"publicationDate":"2026-08-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148798246","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}