Yet H Khor, Catharina C Moor, Wolfgang Merkt, Katharina Buschulte, Boyang Zheng, Veronika Müller, Michael Kreuter
{"title":"Treating connective tissue disease-associated interstitial lung disease - think outside the box: a perspective.","authors":"Yet H Khor, Catharina C Moor, Wolfgang Merkt, Katharina Buschulte, Boyang Zheng, Veronika Müller, Michael Kreuter","doi":"10.1183/16000617.0046-2025","DOIUrl":"10.1183/16000617.0046-2025","url":null,"abstract":"<p><p>Connective tissue disease-associated interstitial lung disease is one of the most common subtypes of interstitial lung disease, which is a leading cause of morbidity and mortality in patients with these systemic autoimmune rheumatic diseases. A spectrum of disease trajectories exists within individual and across different connective tissue diseases. In individuals with connective tissue diseases who are at risk or at the early asymptomatic stage with interstitial lung changes, we have potential windows of opportunity for interventions to prevent the development of or progression to interstitial lung disease. In this perspective, we use systemic sclerosis and rheumatoid arthritis as sample cases to discuss emerging knowledge on disease pathogenesis, as well as to apply the preventative medicine concept for pharmacotherapeutic approaches at different disease stages of connective tissue disease-associated interstitial lung disease.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":10.4,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12284716/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144697982","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}
Natalia El-Merhie, Claudia A Staab-Weijnitz, Janette K Burgess, Grazyna Kwapiszewska
{"title":"Beyond classical collagen: basement membrane collagen IV in age-associated lung diseases.","authors":"Natalia El-Merhie, Claudia A Staab-Weijnitz, Janette K Burgess, Grazyna Kwapiszewska","doi":"10.1183/16000617.0192-2024","DOIUrl":"10.1183/16000617.0192-2024","url":null,"abstract":"<p><p>Chronic lung diseases such as COPD, asthma, idiopathic pulmonary fibrosis (IPF) and pulmonary hypertension are characterised by aberrant remodelling and degradation of the extracellular matrix. This is particularly evident within the basement membrane. Collagen IV, a major component of the basement membrane, is essential for maintaining structural support and regulating cell behaviour. However, disruptions in collagen IV metabolism and basement membrane integrity have been implicated in the pathogenesis of chronic lung diseases, especially in ageing populations where basement membrane turnover is compromised. Cleavage of collagen IV during basement membrane remodelling generates bioactive fragments known as matrikines, which serve as markers of tissue remodelling and potential diagnostic biomarkers. Despite the prominence of collagen IV in the basement membrane, its role in chronic lung diseases remains understudied compared to other collagen types. This review provides a comprehensive exploration of the roles of basement membrane collagen IV and its matrikines in COPD, asthma, IPF and pulmonary hypertension, emphasising their significance beyond classical matrix components. Through an analysis of clinical studies, animal models and <i>in vitro</i> experiments, the contributions of collagen IV to disease pathogenesis and progression are discussed. Furthermore, potential diagnostic and therapeutic implications of targeting collagen IV are outlined. By providing insights into the relationship between collagen IV and chronic lung diseases, this review aims to guide future research and clinical interventions in the field.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":10.4,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12284717/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144697979","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}
Zachary Miller, Laura-Marie Twardowski, Brenda F Reader, Mauricio Rojas, Mareike Lehmann, Ana L Mora
{"title":"Mechanisms and markers of lung ageing in health and disease.","authors":"Zachary Miller, Laura-Marie Twardowski, Brenda F Reader, Mauricio Rojas, Mareike Lehmann, Ana L Mora","doi":"10.1183/16000617.0233-2024","DOIUrl":"10.1183/16000617.0233-2024","url":null,"abstract":"<p><p>Ageing significantly impacts lung function and increases susceptibility to chronic lung diseases. The lung is a complex organ with multiple cell types that undergo cellular age-related perturbations or hallmarks. As knowledge of ageing mechanisms has progressed, we have a better understanding how intracellular adaptations impact cellular crosstalk and integrate to increase the susceptibility to age-related diseases in the lung. Herein, we discuss the prospects of exhaustion of lung progenitor cells, disrupted lung cell plasticity, perturbation in fibroblasts, impaired adaptive immune responses and alterations in lung microenvironment in the promotion of ageing and age-related lung diseases. Additionally, the ageing process trajectory of the lung depends on a combination of biological, genetic, metabolic, biomechanical and sociobehavioural factors that range from protective phenotypes to accelerated ageing phenotypes. We propose the concept of AgEnOmics, which expands the temporal dimension of lung ageing by distinguishing between chronological ageing and accelerated lung ageing phenotypes. Based on this concept, we define biomarkers of biological ageing that will help to define accelerated ageing and early interventions in biological ageing-related lung diseases.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":10.4,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12284718/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144697981","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}
Nicolas Guibert, Pascalin Roy, Valentin Héluain, Gavin Plat, Juliette Edme, Thomas Villeneuve, Hervé Dutau, Thomas R Gildea
{"title":"A beginner's guide to using personalised three-dimensional airway stents.","authors":"Nicolas Guibert, Pascalin Roy, Valentin Héluain, Gavin Plat, Juliette Edme, Thomas Villeneuve, Hervé Dutau, Thomas R Gildea","doi":"10.1183/16000617.0268-2024","DOIUrl":"10.1183/16000617.0268-2024","url":null,"abstract":"<p><p>Conventional silicone airway stents are effective in relieving stenoses but are prone to complications such as migration and granulation tissue formation. These complications reduce patients' tolerance and induce unwanted procedures, limiting their overall benefit. Over the past decade, personalised, three-dimensional (3D)-printed silicone stents have emerged as a possible solution to some of these concerns. In this narrative review, the authors aim to guide the physician into understanding the relatively straightforward creative process behind 3D stents and the selection process of the best patients for their use. Current use is limited to complex anatomical airway stenoses, but more indications could blossom from future trials as technology, expertise and access develop going forward.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12288983/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144697978","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":"Exercise and pulmonary embolism: a systematic review of exercise safety, feasibility and effectiveness.","authors":"Wu Xiang, Yuan-Jiao Lei, Han Xiang, Ying-Bo Qiu, Jun-Yu Wang, An-Ren Zhang","doi":"10.1183/16000617.0241-2024","DOIUrl":"10.1183/16000617.0241-2024","url":null,"abstract":"<p><strong>Background: </strong>Long-term survivors of pulmonary embolism (PE) exhibit decreased exercise capacity, dyspnoea and a diminished quality of life. Exercise may represent a beneficial strategy for ameliorating persistent symptoms following PE.</p><p><strong>Research question: </strong>Is exercise training beneficial for improving exercise capacity and quality of life in patients with PE? Additionally, is it safe and feasible?</p><p><strong>Study design and methods: </strong>The aim of this systematic review was to evaluate the safety, feasibility and efficacy of exercise training in improving exercise capacity and quality of life in patients with PE. In order to comprehensively assess the available evidence, we conducted a systematic review using a combination of free-text terms and medical subject headings according to database requirements in PubMed, Medline, Web of Science, Scopus, Embase and the Cochrane Library from inception until 17 September 2024.</p><p><strong>Results: </strong>We included a total of nine trials including 583 patients, including 391 in the interventional group and 190 in the control group. The difference in the average adverse event rates between the exercise group (0.5%) and the control group (0%) was not significant. The overall recruitment rate was approximately 51% (range: 38-65%), the withdrawal rate was approximately 5% (range: 0-13%) and the adherence rate was 87% (range: 61-100%). The studies reported average improvements in peak oxygen consumption (exercise group: 7.55 mL·kg<sup>-1</sup>·min<sup>-1</sup>; control group: 1.95 mL·kg<sup>-1</sup>·min<sup>-1</sup>), incremental shuttle walk test distance (exercise group: 142 m; control group: 69.5 m), vitality scores (exercise group: 13.95; control group: 3.95), and role emotional scores (exercise group: 12.05; control group: -0.1). However, due to considerable discrepancies in the scoring systems, an average improvement in Pulmonary Embolism Quality of Life questionnaire score could not be determined. Notably, no improvement in dyspnoea was reported.</p><p><strong>Conclusion: </strong>This systematic review indicates that exercise training seems to be safe and feasible for patients with PE. It appears to enhance patients' exercise capacity and quality of life, although its impact on alleviating dyspnoea remains limited. However, given the absence of large-scale randomised controlled trials, these findings should be interpreted with caution.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":10.4,"publicationDate":"2025-07-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12284715/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144697980","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":"Immune responses of different hosts to <i>Pneumocystis</i> infection.","authors":"Jun Li, Xiangdong Mu, Haichao Li, Xinmin Liu","doi":"10.1183/16000617.0247-2024","DOIUrl":"10.1183/16000617.0247-2024","url":null,"abstract":"<p><p><i>Pneumocystis</i> pneumonia constitutes a critical life-threatening opportunistic infection, where the host's immune response plays a central role in its pathogenesis. Immunocompetent individuals are typically capable of eradicating <i>Pneumocystis</i> without exhibiting clinical symptoms. In contrast, individuals with compromised immune systems are vulnerable to developing <i>Pneumocystis</i> pneumonia, which can lead to severe inflammatory responses and consequent pulmonary damage. This review examines the roles of innate immunity, particularly macrophages and adaptive immunity, including CD4<sup>+</sup> and CD8<sup>+</sup> T-cells, as well as key cytokines, in the defence against <i>Pneumocystis</i> infection across various host categories, namely immunocompetent individuals, those infected with HIV and non-HIV-infected individuals, especially those undergoing corticosteroid therapy. By integrating findings from animal models and clinical studies, this review seeks to enhance our understanding of the pathogenesis of <i>Pneumocystis</i> infection across varied immunological contexts.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12238876/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144599791","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}
Jennifer Mann, Hayley Barnes, Jeremy Lew, Yet H Khor, Nicole Goh, Anne E Holland
{"title":"Patient-reported outcome measures for cough used in interstitial lung disease: a systematic review.","authors":"Jennifer Mann, Hayley Barnes, Jeremy Lew, Yet H Khor, Nicole Goh, Anne E Holland","doi":"10.1183/16000617.0206-2024","DOIUrl":"10.1183/16000617.0206-2024","url":null,"abstract":"<p><strong>Introduction: </strong>Validated and reliable patient-reported outcome measures (PROMs) are recommended to assess the severity and impact of cough in interstitial lung disease (ILD). We systematically reviewed the literature to identify PROMs for cough in ILD, examining their psychometric properties.</p><p><strong>Methods: </strong>We searched four databases from inception to 10 January 2025. English-language original articles that described the use of a PROM to measure cough in adults with ILD and addressed the psychometric properties, method of administration or results of usability testing were selected.</p><p><strong>Results: </strong>21 PROMs were evaluated in 35 studies, including 14 in idiopathic pulmonary fibrosis (IPF) and seven in other ILDs, eight cough-specific PROMs, and 13 disease-specific PROMs with a domain for cough. No tool had sufficient evidence for more than 5/7 of the psychometric properties evaluated. There was evidence for content validity for four PROMs in IPF (A Tool to Assess Quality of Life in Idiopathic Pulmonary Fibrosis (ATAQ-IPF), the Cough and Sputum Assessment Questionnaire (CASA-Q), Evaluating Respiratory Symptoms: COPD (E-RS™:COPD) and the Living with Idiopathic Pulmonary Fibrosis Questionnaire (L-IPF)). Only one study evaluated convergent validity using objective cough monitoring, demonstrating high validity for the Leicester Cough Questionnaire (LCQ) (r=-0.74- -0.80) and cough visual analogue scale (VAS) (r=0.80). Acceptable internal consistency (α>0.7) was demonstrated for 10 PROMs (ATAQ-IPF, the Cross-Atlantic modification of ATAQ-IPF, the Chinese version of ATAQ-IPF, CASA-Q, E-RS™:COPD, LCQ, L-IPF, the IPF-specific version of St George's Respiratory Questionnaire (SGRQ), the modified version of the Edmonton System Assessment System and SGRQ). The cough VAS demonstrated good predictive validity and L-IPF was responsive to ILD-specific therapies, with effect sizes ranging from 0.32 to 0.44.</p><p><strong>Conclusion: </strong>Evidence supporting the measurement properties of available PROMs for cough in ILD is limited. Further validation of existing instruments and the development of new disease-specific PROMs are needed.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12238875/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144599792","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}
Rosa Faner, Michael H Cho, Gerard H Koppelman, Erik Melén, Stijn E Verleden, Shyamali C Dharmage, Silke Meiners, Alvar Agusti
{"title":"Towards early detection and disease interception of COPD across the lifespan.","authors":"Rosa Faner, Michael H Cho, Gerard H Koppelman, Erik Melén, Stijn E Verleden, Shyamali C Dharmage, Silke Meiners, Alvar Agusti","doi":"10.1183/16000617.0243-2024","DOIUrl":"10.1183/16000617.0243-2024","url":null,"abstract":"<p><p>COPD is \"a heterogeneous lung condition characterized by chronic respiratory symptoms due to abnormalities of the airways and/or alveoli that cause persistent, often progressive, airflow obstruction\". COPD has been traditionally associated with tobacco smoking and accelerated lung function decline. However, our understanding of the pathogenesis of COPD has changed significantly over the past few years due to the recognition that different lung function trajectories starting in early life and progressing across the lifespan are also important pathways to COPD. Further, today, it is well accepted that there are multiple genetic, host and environmental factors (<i>i.e.</i>, aetiotypes) that can cause COPD and contribute to its clinical heterogeneity. Here, we review current understanding of the environmental, genomic and immune factors associated with the early-life origins of COPD. We also discuss the current knowledge gaps and how this new knowledge can facilitate earlier detection and disease interception of COPD across the lifespan, thus reducing its disease burden and improving the well-being and prognosis of COPD patients.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12238877/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144599794","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}
Craig A Williams, Brenda Button, Tiffany J Dwyer, Elpis Hatziagorou, Kelly A Mackintosh, Melitta A McNarry, Dewi Paris, James Shelley, Helge Hebestreit, Judy Bradley, Mayara Silveira Bianchim
{"title":"The 2025 European Cystic Fibrosis Society position statement on physical activity assessment in cystic fibrosis.","authors":"Craig A Williams, Brenda Button, Tiffany J Dwyer, Elpis Hatziagorou, Kelly A Mackintosh, Melitta A McNarry, Dewi Paris, James Shelley, Helge Hebestreit, Judy Bradley, Mayara Silveira Bianchim","doi":"10.1183/16000617.0279-2024","DOIUrl":"10.1183/16000617.0279-2024","url":null,"abstract":"<p><strong>Background: </strong>Recent advances in the measurement of physical activity have significantly enhanced the analyses and interpretation in relation to health and well-being. Thus, we sought to revise and expand the 2015 position statement on the measurement of physical activity and provide guidance to clinicians and researchers for measuring physical activity in cystic fibrosis (CF) clinical practice and research.</p><p><strong>Methods: </strong>This study was registered with the International Prospective Register of Systematic Review (PROSPERO) database (CRD42022292165). Three databases (Medline, Embase and Cumulative Index to Nursing and Allied Health Literature) were searched for studies investigating the measurement of physical activity and sedentary time in people with CF irrespective of age or duration. The Quality Assessment for Diverse Studies was used to assess methodological concern. A mixed-methods framework synthesis was used to extract, map, chart, categorise and aggregate study findings.</p><p><strong>Results: </strong>In total, 7439 potentially relevant publications were identified. Following screening of titles and abstracts, 422 full texts were retrieved and assessed for eligibility, with 90 studies included. There was considerable variation in the methods of assessment, data processing and analytical interpretation of data.</p><p><strong>Conclusion: </strong>It is recommended that device-based physical activity metrics are presented as time spent in different intensity categories (<i>e.g.</i>, light, moderate and vigorous) and to include sedentary and sleep time. For data analysis, the data resolution should be at least 1 s (minimum 30 Hz) to enable clinical teams to obtain representative categorisation of patients' physical activity patterns. Validated questionnaires (<i>e.g.</i>, the Habitual Activity Estimation Scale) offer additional opportunities to assess physical activity, whilst diaries can add context but should be viewed as secondary outcome measurements.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12249234/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144599793","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":"Clinical remission in chronic rhinosinusitis with nasal polyps with biologics: a premature promise?","authors":"Matteo Gelardi, Rossana Giancaspro","doi":"10.1183/16000617.0093-2025","DOIUrl":"10.1183/16000617.0093-2025","url":null,"abstract":"","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 177","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12238874/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144599790","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}