Thorax最新文献

{"title":"Holistic management of symptom burden in fibrosing interstitial lung diseases (F-ILDs).","authors":"Rachana Krishna, Kathleen Lindell","doi":"10.1136/thorax-2024-222056","DOIUrl":"https://doi.org/10.1136/thorax-2024-222056","url":null,"abstract":"<p><strong>Background: </strong>Disease modifying therapies for progressive fibrotic interstitial lung diseases (F-ILDs) slow physiological decline but have not consistently shown to improve patient-reported symptoms or health-related quality of life. Patients with F-ILD experience a substantial symptom burden that necessitates comprehensive supportive care alongside antifibrotic treatment. This review summarises the current evidence for management strategies for F-ILD to be considered in conjunction with disease-modifying therapies.</p><p><strong>Symptom management: </strong>Dyspnoea, chronic cough, fatigue, anxiety and depression are highly prevalent in F-ILD and significantly impair daily functioning. Dyspnoea management includes non-pharmacological interventions, personalised self-management strategies and selective use of low dose opioids in advanced disease though overall benefit may be modest. Chronic cough may be addressed through behavioural and speech therapy, treatment of contributing comorbidities such as gastro-oesophageal reflux disease and antitussive therapies, including neuromodulators and low dose opioids for refractory symptoms. Fatigue and psychological distress require routine screening, evaluation of modifiable factors and targeted interventions.</p><p><strong>Oxygen therapy: </strong>Oxygen therapy remains a standard of care for patients with resting or exertional hypoxaemia. While it improves oxygenation, its effect on dyspnoea and symptoms is variable, underscoring the importance of individualised assessment and patient-centred decision making.</p><p><strong>Pulmonary rehabilitation: </strong>Pulmonary rehabilitation provides evidence-based improvements in exercise capacity, symptom burden and health-related quality of life. It also offers important psychological benefits and should be integrated early and maintained as feasible throughout the disease course.</p><p><strong>Palliative care: </strong>Given the unpredictable trajectory of F-ILD, early integration of palliative care is essential. Discussions regarding prognosis, advance care planning and end of life preferences, supported by a multidisciplinary care model, enable holistic, goal concordant care across the progressive disease trajectory. A multidisciplinary care model is beneficial for providing individualised, holistic care throughout the patient's progressive disease trajectory.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148211046","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":"Paediatric bronchiectasis phenotypes and their association with clinical outcomes: development and validation in two prospective clinical cohorts.","authors":"Vikas Goyal, Don Vicendese, Keith Grimwood, Stephanie Yerkovich, Shyamali Dharmage, Anne B Chang","doi":"10.1136/thorax-2025-224359","DOIUrl":"https://doi.org/10.1136/thorax-2025-224359","url":null,"abstract":"<p><strong>Background: </strong>In most chronic airway diseases, identifying phenotypes has advanced clinical practice and research. However, no such phenotypes exist for paediatric bronchiectasis. We therefore sought to develop robust paediatric bronchiectasis phenotypes and to investigate whether they were associated with relevant clinical outcomes.</p><p><strong>Methods: </strong>Latent class analysis was independently applied to two study cohorts of children with bronchiectasis. Data from 158 children from one study (BEST, development cohort) were used to identify phenotypes and their associations with relevant clinical outcomes. A cohort of 198 children from a second study (BAMP) was used to validate these phenotypes and compare findings for consistency.</p><p><strong>Results: </strong>A three-class model was the best fit in the BEST cohort. Based on clinical characteristics, we labelled the phenotypes as 'Baseline-Well', 'Wheeze-Dyspnoea Predominant' and 'Daily Wet-Cough Predominant'. These phenotypes were validated in the BAMP cohort. In both development and validation cohorts, the 'Daily Wet-Cough Predominant' phenotype was associated with higher numbers of bronchiectatic lobes (OR 1.61, 95% CI 0.61 to 4.27; and 3.06, 95% CI 1.30 to 7.21 for BEST and BAMP cohorts, respectively) and more bronchiectasis-related hospitalisations ever at enrolment (incidence rate ratio (IRR) 4.33, 95% CI 1.94 to 9.66; and 2.96, 95% CI 1.07 to 8.20, respectively) and also within 2 years of enrolment (IRR 2.68, 95% CI 1.24 to 5.8; and 1.54, 95% CI 0.72 to 3.28, respectively) than the reference phenotype, although the strengths of evidential support differed.</p><p><strong>Conclusions: </strong>We identified and validated three novel paediatric bronchiectasis phenotypes, and linked them to distinct clinical outcomes. These phenotypes might enable targeted interventions and improve participant selection for clinical trials.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148206185","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":"Guideline-based prognostic factors associated with mortality in pulmonary embolism: a systematic review and meta-analysis.","authors":"Kevin Durr, Bram Rochwerg, Shannon M Fernando, John Granton, Daniel Brodie, Roberto Lorusso, Hilary Meggison, Akira Kuriyama, Deborah Siegal, Marc Carrier, Philip S Wells, Ewout W Steyerberg, Alexandre Tran","doi":"10.1136/thorax-2025-223933","DOIUrl":"10.1136/thorax-2025-223933","url":null,"abstract":"<p><strong>Introduction: </strong>Optimal management for patients with acute pulmonary embolism (PE) at elevated risk of decompensation remains unclear given the lack of predictors and the limitations of existing risk stratification tools. Thus, we aimed to evaluate the prognostic association between clinical variables and short-term mortality in patients with acute PE.</p><p><strong>Methods: </strong>Medline and EMBASE were searched from inception through 19 May 2025. We included English-language studies that described adult inpatients with a confirmed acute PE and evaluated prognostic factors associated with short-term mortality. Two authors performed citation screening and data extraction. Only adjusted OR were included in the data synthesis. We performed pooling using a random-effects model and inverse variance weighting. Risk of bias was evaluated using the Quality in Prognosis Studies tool. We assessed certainty of evidence using the Grading of Recommendations, Assessment, Development, and Evaluations approach.</p><p><strong>Results: </strong>We included 70 studies involving 1 446 190 participants in our review. Variables with a moderate or high certainty association with short-term mortality in acute PE include increasing age, an elevated Pulmonary Embolism Severity Index (PESI) Score, an abnormal simplified PESI Score, an elevated heart rate, a reduced systolic blood pressure, a lower oxygen saturation, a higher respiratory rate, active malignancy, heart failure, chronic lung disease, an elevated troponin, a larger right ventricle (RV) to left ventricle ratio and evidence of RV dysfunction.</p><p><strong>Discussion: </strong>We identified multiple variables associated with early mortality in acute PE. Notably, we demonstrate that RV function, a marker omitted from commonly used risk stratification tools, represents an important prognostic variable that is supported by multiple society guidelines.</p><p><strong>Registration: </strong>We registered the protocol with the Open Science Framework (https://osf.io/9w2jg).</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147975731","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":"Trends in asthma incidence and prevalence in 2 million children and adolescents in Catalonia, Spain, 2010-2024: a population-based cohort study.","authors":"Anna Palomar-Cros, Alicia Abellan, Berta Raventós, Andrea Pistillo, Carlen Reyes, Agustina Guiliodori, Laura Granés, Juan Luís García Rivero, Katia Mc Verhamme, Elena Roel, Talita Duarte-Salles","doi":"10.1136/thorax-2026-224985","DOIUrl":"https://doi.org/10.1136/thorax-2026-224985","url":null,"abstract":"<p><strong>Background: </strong>Asthma represents a major public health concern in childhood and adolescence. However, population-based estimates of trends in its epidemiology in Spain are limited.</p><p><strong>Methods: </strong>We conducted a population-based cohort study using primary care electronic health records in Catalonia, Spain, from 2010 to 2024. Asthma diagnoses, ascertained through Systematized Nomenclature of Medicine (SNOMED) codes, were leveraged to estimate annual incidence rates (IR) and prevalence among children aged 1-17 years. Results were stratified by sex, age and Socioeconomic Deprivation Index (SDI). Incidence rate ratios (IRR) were estimated to investigate differences in overall incidence of asthma in these sociodemographic groups. Joinpoint modelling was used to investigate time trends in incidence.</p><p><strong>Results: </strong>We included 2 001 625 individuals (48.40% females, median age 7 years). Crude IR of asthma (2010-2024) was 687.46 (95% CI 683.09 to 691.85) per 100 000 person-years. Incidence was highest in males (IRR=1.35, 95% CI 1.33 to 1.37, vs females) until adolescence, after which the pattern reversed, youngest individuals (IRR=3.03 95% CI 2.96 to 3.11, for 1-4 versus 15-17) and in individuals living in the most deprived areas (IRR=1.14, 95% CI 1.11 to 1.16, vs least deprived SDI quintile). Incidence declined before the COVID-19 pandemic, dropped sharply during it, and then increased among children aged 5-14 years post-pandemic, while consistently decreased in children aged 1-4 years. Asthma prevalence showed an upward trend until 2019 followed by stabilisation until 2024, when its prevalence was 7.36% (7.3%-7.41%).</p><p><strong>Conclusion: </strong>Asthma remains a major public health burden in children and adolescents, with incidence and prevalence varying by age, sex and socioeconomic status, making continuous monitoring essential to guide prevention.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148206256","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":"Caught between breath and movement: should we recommend outdoor activity for patients with COPD in polluted environments?","authors":"Jiawei Zhang, Zorana Jovanovic Andersen","doi":"10.1136/thorax-2026-225257","DOIUrl":"https://doi.org/10.1136/thorax-2026-225257","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148206187","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":"Establishing the top 10 research priorities for lung volume reduction treatment for people with COPD.","authors":"Sara C Buttery, Richard Barraclough, Timothy J P Batchelor, Sofina Begum, Rocco Bilancia, Edward J Caruana, James W Dodd, Justin L Garner, Neil J Greening, Samuel V Kemp, Alan J B Kirk, Rod A Lawson, Babu Naidu, Christopher M Orton, Karen Redmond, Carole Ridge, Pallav L Shah, Jane S Watson, Nicholas S Hopkinson","doi":"10.1136/thorax-2026-224791","DOIUrl":"https://doi.org/10.1136/thorax-2026-224791","url":null,"abstract":"<p><p>Lung volume reduction procedures are an established evidence-based aspect of chronic obstructive pulmonary disease (COPD) care. We conducted a research prioritisation exercise involving people with COPD and healthcare professionals across a range of disciplines, to identify a clear set of 10 questions to guide the development of research proposals in this area. Priorities were identified using an iterative approach based on the James Lind Alliance methodology. The final set of 10 priorities address the identification, assessment and optimisation of patients with COPD prior to any procedure, how lung volume reduction procedures are conducted and how care after the procedure has taken place should be organised.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148176832","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":"Periodic breathing at altitude is an adaptive response: lessons for similar conditions at sea level.","authors":"Matthew T Naughton","doi":"10.1136/thorax-2026-225168","DOIUrl":"https://doi.org/10.1136/thorax-2026-225168","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148164648","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":"When the reliever becomes the risk.","authors":"Chloe I Bloom","doi":"10.1136/thorax-2026-225211","DOIUrl":"https://doi.org/10.1136/thorax-2026-225211","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148164523","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":"Preterm birth, disrupted lung growth and the road to COPD.","authors":"Dinh Son Bui, Jiaxin Li, Shyamali Dharmage","doi":"10.1136/thorax-2026-225167","DOIUrl":"https://doi.org/10.1136/thorax-2026-225167","url":null,"abstract":"","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148164681","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":"Survival in congenital central hypoventilation syndrome (CCHS): data from the European CCHS Consortium.","authors":"Plamen Bokov, Panagiota Chaitidou-Kolb, Benjamin Dudoignon, Maria Giovanna Paglietti, Nuria Madureira, Maria Angeles Garcia-Teresa, Christophe Delclaux, Martin Samuels","doi":"10.1136/thorax-2025-224512","DOIUrl":"https://doi.org/10.1136/thorax-2025-224512","url":null,"abstract":"<p><strong>Introduction: </strong>Congenital central hypoventilation syndrome (CCHS) is a rare disorder characterised by autonomic dysfunction and impaired respiratory control. While advances in ventilatory support have improved survival, risk factors for mortality remain incompletely understood. Our objective was to investigate the impact of genetic and clinical parameters on mortality in children and young adults with genetically confirmed CCHS.</p><p><strong>Methods: </strong>Data were collected from the European CCHS Consortium registry, encompassing patient information collected from 2012 to 2021. This dataset included information for 240 patients with genetically confirmed CCHS. Complete outcome data were available for 211 patients. Survival outcomes were analysed using Kaplan-Meier curves and Cox proportional hazards regression models.</p><p><strong>Results: </strong>The overall mortality rate was 14% (95% CI 9% to 19%), with most deaths occurring in early childhood. The 25-year survival probability was 89% (95% CI 79% to 100%) for children and young adults with isolated CCHS, compared with 26% (95% CI 6% to 100%) for those with CCHS and Hirschsprung disease (HSCR), corresponding to a 6.8-fold increased risk of mortality (95% CI 2.2 to 21.1) associated with HSCR. In multivariable Cox models including genotype and HSCR as covariates, mortality risk was consistently associated with HSCR. While respiratory signs at initial presentation were comparable between groups, children with both CCHS and HSCR exhibited signs of more severe autonomic dysfunction, requiring more frequent resuscitation and intubation at birth.</p><p><strong>Conclusions: </strong>HSCR is a key determinant of increased mortality in children with CCHS, likely because of its association with more extensive autonomic dysfunction.</p>","PeriodicalId":23284,"journal":{"name":"Thorax","volume":" ","pages":""},"PeriodicalIF":7.7,"publicationDate":"2026-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148164108","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}