Lianne Ten Have, Fleur L Meulmeester, Kim de Jong, Anneke Ten Brinke
{"title":"Patient-centred outcomes in severe asthma: fatigue, sleep, physical activity and work.","authors":"Lianne Ten Have, Fleur L Meulmeester, Kim de Jong, Anneke Ten Brinke","doi":"10.1183/16000617.0122-2024","DOIUrl":"10.1183/16000617.0122-2024","url":null,"abstract":"<p><p>Severe asthma places a significant burden on patients, with recent research revealing overlooked patient needs extending beyond physical symptoms. To optimise the patient-centred approach to managing severe asthma, it is crucial to deepen our understanding of these needs. This review examines the prevalence and impact of four prioritised patient needs in severe asthma, namely fatigue, sleep disturbances, physical inactivity and reduced presence and productivity at work. It explores how these factors relate to classic asthma outcomes and quality of life, and the potential impact of interventions. Fatigue affects up to 90% of patients, while sleep difficulties impact 70-75% of severe cases, contributing to impaired daily function and quality of life. Although both are linked to asthma control, the cause-and-effect relationship remains unclear, making it clinically intriguing to investigate whether interventions targeting fatigue or sleep problems affect asthma control. In asthma patients, physical inactivity occurs both as consequence and contributing factor to uncontrolled disease. Interventions promoting physical activity improve asthma control and quality of life, suggesting a potential role in severe asthma management. Finally, work productivity loss, notably present in severe asthma cases, strongly correlates with asthma control and exacerbations. While biologic therapies show potential to reverse this loss, their effects on physical activity, fatigue and sleep disturbances warrant further investigation. Nonpharmacological interventions targeting these needs, such as pulmonary rehabilitation and behavioural therapies, may provide opportunities to enhance patients' well-being. Overall, this review highlights significant gaps in understanding patient-centred aspects of severe asthma, urging for research on comprehensive interventions to improve patients' lives.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11880904/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143566398","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}
Zainab Ahmadi, Natasha E Smallwood, Anne-Marie Russell, Ravijyot Saggu, Lorena Romero, Anne E Holland, Magnus Ekström
{"title":"Supplemental oxygen for symptomatic relief in people with serious respiratory illness: a systematic review and meta-analysis.","authors":"Zainab Ahmadi, Natasha E Smallwood, Anne-Marie Russell, Ravijyot Saggu, Lorena Romero, Anne E Holland, Magnus Ekström","doi":"10.1183/16000617.0025-2024","DOIUrl":"10.1183/16000617.0025-2024","url":null,"abstract":"<p><strong>Background: </strong>People with serious respiratory illness frequently have a high symptom burden and may be prescribed supplemental oxygen therapy with the aims of reducing the severity of breathlessness and improving health-related quality of life (HRQoL). This systematic review and meta-analysis aimed to assess the effectiveness of oxygen therapy <i>versus</i> no oxygen on 1) breathlessness, 2) HRQoL and 3) adverse events.</p><p><strong>Methods: </strong>A comprehensive search was performed in Embase, Medline and the Cochrane Central Register of Controlled Trials for randomised controlled trials published prior to June 2022. We used the Cochrane Risk of Bias Tool for appraising the studies and conducted random-effect meta-analyses when appropriate. We pooled effects recorded on different scales as standardised mean differences (SMDs) with 95% confidence intervals. Lower SMDs indicated decreased breathlessness or HRQoL. We assessed the certainty of evidence using the Grading of Recommendations, Assessment, Development and Evaluation framework.</p><p><strong>Results: </strong>We found that supplemental oxygen (compared with sham air or no treatment), reduced breathlessness intensity during laboratory exercise testing (SMD -0.75, 95% CI -1.23--0.28, 12 randomised control trials (RCTs), 245 participants), but had no shown effect on breathlessness measured in daily life (SMD -0.08, 95% CI -0.41-0.26, one RCT, 213 participants) or HRQoL (SMD -0.06, -0.17-0.05, 14 RCTs, 1062 participants). Few or no adverse events related to oxygen therapy were reported. For all the outcomes, the certainty of evidence was low.</p><p><strong>Conclusions: </strong>Oxygen improved exertional breathlessness in laboratory-based exercise studies but was not shown to improve breathlessness or HRQoL in daily life.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11880903/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143566401","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":"Lack of diversity in antifibrotic trials for pulmonary fibrosis: a systematic review.","authors":"Amy Pascoe, Xinye Esther Chen, Natasha Smallwood","doi":"10.1183/16000617.0201-2024","DOIUrl":"10.1183/16000617.0201-2024","url":null,"abstract":"<p><strong>Introduction: </strong>Social determinants of health (SDH), including age, sex, ethnicity, socioeconomic status and rurality, influence health outcomes. Clinical trials investigating antifibrotic agents for people with idiopathic pulmonary fibrosis (IPF) have been conducted in predominantly White and male populations; it is unclear whether other SDH have been considered. This study aimed to investigate active consideration and reporting of SDH in clinical trials of antifibrotic agents for people with IPF.</p><p><strong>Methods: </strong>Three registries (ClinicalTrials.gov, ANZCTR and International Standard Randomised Controlled Trial Number (ISRCTN)) plus CENTRAL (Cochrane Central Register of Controlled Trials) were searched for clinical trials investigating antifibrotic agents for people with IPF or various progressive fibrotic ILD variants registered from 1 January 2000 until 3 September 2023. Data were extracted regarding trial phase/status, recruitment strategies and eligibility criteria. If trial results were available, SDH data from demographics and subgroup analyses were extracted.</p><p><strong>Results: </strong>Of 313 records identified, 70 trials were included. The majority of trials were phase II or III (77%), 56% were completed and 61% had reported results that included eight terminated trials. All 70 trials specified age and sex, but not other SDH, within their eligibility criteria. Of 43 trials reporting results, all reported age and sex and 40 (95%) reported ethnicity. 10 387 participants were described (74% male, 77% White, 16% Asian and <1% Black). Descriptors for ethnicity varied considerably. Five trials (12%) included only White participants and three (7%) included only Asian participants. No other SDH were reported.</p><p><strong>Conclusions: </strong>SDH beyond age, sex and ethnicity were neither considered nor reported in antifibrotic IPF trials. Trial populations were predominantly male and White. There is a need to actively consider SDH to ensure diverse and representative clinical trial populations.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11880902/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143566395","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}
Latisha E Witjaksono, Max Schulte, Anne E Holland, Marlies S Wijsenbeek, Yet H Khor
{"title":"Cough monitoring systems in adults with chronic respiratory diseases: a systematic review.","authors":"Latisha E Witjaksono, Max Schulte, Anne E Holland, Marlies S Wijsenbeek, Yet H Khor","doi":"10.1183/16000617.0212-2023","DOIUrl":"10.1183/16000617.0212-2023","url":null,"abstract":"<p><strong>Background: </strong>The role of objective cough monitoring systems for assessments in adults with chronic respiratory diseases (CRDs) is unclear. This systematic review aimed to synthesise current literature on frequency of use and characteristics of these systems.</p><p><strong>Methods: </strong>MEDLINE, Embase and CENTRAL were systematically searched to identify relevant literature evaluating cough in adults with CRDs using objective cough monitoring systems. The primary outcomes were utility and characteristics of the systems, with the secondary outcome being usability.</p><p><strong>Results: </strong>We identified 54 primary studies (4909 patients, with 3364 having idiopathic chronic cough). Included studies were generally of low risk of bias. Objective monitoring systems identified were VitaloJAK (n=19 studies), Leicester Cough Monitor (LCM, n=18), LEOSound (n=2), PulmoTrack (n=2), Hull Automated Cough Counter (HACC, n=1), LifeShirt (n=1), and unnamed devices (n=11). There was limited assessment against manual counting, with low-to-moderate correlation to patient-reported outcome measures for VitaloJAK (p<0.05), LCM (r=0.43-0.78) and unnamed devices (r=0.38-0.40). Test-retest consistency was evaluated in two studies, showing favourable results. There was at least moderate effect size of longitudinal measurement changes to various treatments for VitaloJAK (nine out of 16), LCM (two out of eight), HACC (n=1), LCM and HACC (n=1), PulmoTrack (n=1) and unnamed devices (n=3).</p><p><strong>Conclusions: </strong>Few studies evaluated the agreement of objective cough monitoring systems against manual counting. Most studies were conducted in patients with idiopathic chronic cough, with the VitaloJAK and LCM being were the most evaluated objective cough monitoring systems. Further evaluation of objective cough monitoring systems is needed for research and clinic application.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11880901/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143566420","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}
Lenn Maessen, Leonoor S Boers, Jannes Heylen, Frank van Someren Gréve, Joost Wauters, Lieuwe D J Bos, Simon Feys
{"title":"Viral reactivations and fungal infections in nonresolving acute respiratory distress syndrome.","authors":"Lenn Maessen, Leonoor S Boers, Jannes Heylen, Frank van Someren Gréve, Joost Wauters, Lieuwe D J Bos, Simon Feys","doi":"10.1183/16000617.0153-2024","DOIUrl":"10.1183/16000617.0153-2024","url":null,"abstract":"<p><p>Acute respiratory distress syndrome (ARDS) is a condition affecting 10% of patients requiring admission to the intensive care unit and results from endothelial dysfunction, alveolar epithelial injury and unbalanced inflammation, leading to exudative pulmonary oedema. A significant portion of these patients experience a lung injury that fails to resolve. Persistent or worsening respiratory failure beyond 5 days after the initiation of mechanical ventilation is referred to as nonresolving ARDS. Viral and fungal pathogens can exploit the hyperinflammatory environment and altered immune landscape in ARDS, perpetuating a cycle of ongoing inflammation and lung injury, thereby contributing to the progression towards and persistence of nonresolving ARDS, even in previously immunocompetent patients. This review discusses the significance, pathophysiology, diagnostic challenges and key knowledge gaps concerning various viral and fungal pathogens in nonresolving ARDS, with a particular focus on influenza-associated and COVID-19-associated pulmonary aspergillosis and pulmonary reactivation of <i>Herpesviridae</i>, such as cytomegalovirus and herpes simplex virus. Diagnosing these infections is challenging due to their nonspecific clinical presentation and the inability of current tests to distinguish between fungal colonisation or asymptomatic viral shedding and clinically significant infections or reactivations. A deeper understanding of the complex interplay between these pathogens and the host immune system in the context of ARDS, combined with advances in diagnostic and therapeutic strategies, has the potential to enhance the management and prognosis of patients with nonresolving ARDS.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11836671/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457359","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}
Sarah Haesevoets, Eva Arents, Dries Cops, Kirsten Quadflieg, Maarten Criel, David Ruttens, Marc Daenen, Dieter Stevens, Veerle Surmont, Heleen Demeyer, Chris Burtin
{"title":"The impact of lung surgery, with or without (neo-)adjuvant therapy, on physical functioning in patients with nonsmall cell lung cancer: a scoping review.","authors":"Sarah Haesevoets, Eva Arents, Dries Cops, Kirsten Quadflieg, Maarten Criel, David Ruttens, Marc Daenen, Dieter Stevens, Veerle Surmont, Heleen Demeyer, Chris Burtin","doi":"10.1183/16000617.0156-2024","DOIUrl":"10.1183/16000617.0156-2024","url":null,"abstract":"<p><strong>Background: </strong>Patients with early stage (I-IIIA) nonsmall cell lung cancer (NSCLC) are typically treated <i>via</i> surgery, often accompanied by (neo-)adjuvant therapy. These interventions impose a significant burden on patients and potentially impact their physical functioning (PF). The impact on PF remains uncertain and existing evidence has not yet been systematically outlined.</p><p><strong>Objective: </strong>This scoping review aimed to synthesise evidence concerning the effects of lung surgery, with or without (neo-)adjuvant therapy, on the PF of patients with NSCLC.</p><p><strong>Methods: </strong>PubMed, Web of Science and Cochrane databases were systematically searched from inception until 1 July 2023. A comprehensive framework based on the International Classification of Functioning, Disability, and Health was used to define various aspects of PF. Longitudinal studies, reporting PF prior to and after NSCLC treatment, and cross-sectional studies reporting PF after treatment were included.</p><p><strong>Results: </strong>85 included studies assessed the effects of surgery with (n=7) or without (n=78) (neo-) adjuvant therapy on body function (n=29), activity (n=67) and/or participation (n=15). 98% of reported outcomes within the longitudinal studies indicate a decline in PF, with 52% demonstrating significant deteriorations, with follow-up times ranging from immediately post-operative up to 1 year after treatment. Cross-sectional studies show impaired PF in 71% of reported outcomes.</p><p><strong>Conclusion: </strong>PF of patients with NSCLC tends to deteriorate following lung surgery, irrespective of additional (neo-)adjuvant therapy. While the negative impact of lung surgery on ICF categories of \"body function\" and \"activity\" have been described to some depth, insights into the impact on \"participation\" are lacking.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11836670/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457357","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}
Guiling Zhou, Nina Dael, Stefan Verweij, Spyros Balafas, Sumaira Mubarik, Katrien Oude Rengerink, Anna Maria Gerdina Pasmooij, Debbie van Baarle, Peter G M Mol, Geertruida H de Bock, Eelko Hak
{"title":"Effectiveness of COVID-19 vaccines against SARS-CoV-2 infection and severe outcomes in adults: a systematic review and meta-analysis of European studies published up to 22 January 2024.","authors":"Guiling Zhou, Nina Dael, Stefan Verweij, Spyros Balafas, Sumaira Mubarik, Katrien Oude Rengerink, Anna Maria Gerdina Pasmooij, Debbie van Baarle, Peter G M Mol, Geertruida H de Bock, Eelko Hak","doi":"10.1183/16000617.0222-2024","DOIUrl":"10.1183/16000617.0222-2024","url":null,"abstract":"<p><strong>Background: </strong>Up-to-date evidence from European studies on long-term vaccine effectiveness (VE) of COVID-19 vaccines is lacking. This review aimed to evaluate effectiveness and durability of primary vaccine series and boosters in preventing infection and severe outcomes in the European population.</p><p><strong>Methods: </strong>We conducted systematic searches of PubMed and Embase up to 22 January 2024. We included observational studies that evaluated VE against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection or severe disease (hospitalisation, intensive care unit admission or death) for primary series and boosters in Europe. We applied a random-effects meta-analysis model.</p><p><strong>Results: </strong>We included 33 studies and over 56 million participants. The overall VE of the complete primary series against infection with any SARS-CoV-2 variant was 70.7%. VE was lower for Omicron, at 26.1%, than for pre-Omicron strains, at 77.0%. Over time, VE against infection by any variant decreased from 68.9% to 38.9% after 6 months. Boosters restored VE to 76.4% and maintained at 58.4% after 3 months. The overall VE of a complete primary series for severe outcomes due to any variant was 87.4%, with 93.3% for pre-Omicron and 62.8% for Omicron strains. Protection against severe outcomes declined less than for infection. 6 months after the primary series, the vaccine still provided over 50% protection against severe outcomes caused by Omicron. Boosters restored VE to 87.9% and maintained at 78.5% after 3 months.</p><p><strong>Conclusion: </strong>VE against SARS-CoV-2 infection declines markedly with time and Omicron variants. Protection against severe outcomes was more durable and resistant to viral mutation. Boosters restored protection, emphasising the need for timely booster vaccination for vulnerable populations.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11836669/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457414","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}
Louise Bondeelle, Sophie Clément, Anne Bergeron, Caroline Tapparel
{"title":"Lung stem cells and respiratory epithelial chimerism in transplantation.","authors":"Louise Bondeelle, Sophie Clément, Anne Bergeron, Caroline Tapparel","doi":"10.1183/16000617.0146-2024","DOIUrl":"10.1183/16000617.0146-2024","url":null,"abstract":"<p><p>Stem cells are capable of self-renewal and differentiation into specialised types. They range from totipotent cells to multipotent or somatic stem cells and ultimately to unipotent cells. Some adult multipotent stem cells can have the potential to regenerate and colonise diverse tissues. The respiratory airways and lung mucosa, exposed to ambient air, perform vital roles for all human tissues and organs. They serve as barriers against airborne threats and are essential for tissue oxygenation. Despite low steady-state turnover, lungs are vulnerable to injuries and diseases from environmental exposure. Lung stem cells are crucial due to their regenerative potential and ability to replace damaged cells. Lung repair with extrapulmonary stem cells can occur, leading to the coexistence of respiratory cells with different genetic origins, a phenomenon known as airway epithelial chimerism. The impact of such chimerism in lung repair and disease is actively studied. This review explores different stem cell types, focusing on pulmonary stem cells. It discusses airway epithelium models derived from stem cells for studying lung diseases and examines lung chimerism, particularly in lung transplantation and haematopoietic stem cell transplantation, highlighting its significance in understanding tissue repair and chimerism-mediated repair processes in lung pathology.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11836672/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457415","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}
Pasquale Tondo, Claudia Meschi, Marco Mantero, Giulia Scioscia, Matteo Siciliano, Matteo Bradicich, Giulia M Stella
{"title":"Sex and gender differences during the lung lifespan: unveiling a pivotal impact.","authors":"Pasquale Tondo, Claudia Meschi, Marco Mantero, Giulia Scioscia, Matteo Siciliano, Matteo Bradicich, Giulia M Stella","doi":"10.1183/16000617.0121-2024","DOIUrl":"10.1183/16000617.0121-2024","url":null,"abstract":"<p><p>Sex and gender differences significantly influence lung parenchyma development, beginning as early as the embryonic stages of human life. Although this association is well known in the clinical manifestations of some relevant pulmonary diseases, there is less data available regarding their effects on cell biological programmes across different stages of body development. A deep understanding of these mechanisms could help in defining preventive strategies tailored to a fully personalised approach to respiratory medicine. From this perspective, this review aims to analyse the influence of sex and gender on bronchoalveolar and vascular compartments from embryonic and neonatal stages through to adolescence, adulthood and elder age.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-02-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11836673/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143457356","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}
Jana Dietrich, Alice Kang, Birger Tielemans, Stijn E Verleden, Hassan Khalil, Florian Länger, Philipp Bruners, Steven J Mentzer, Tobias Welte, Michael Dreher, Danny D Jonigk, Maximilian Ackermann
{"title":"The role of vascularity and the fibrovascular interface in interstitial lung diseases.","authors":"Jana Dietrich, Alice Kang, Birger Tielemans, Stijn E Verleden, Hassan Khalil, Florian Länger, Philipp Bruners, Steven J Mentzer, Tobias Welte, Michael Dreher, Danny D Jonigk, Maximilian Ackermann","doi":"10.1183/16000617.0080-2024","DOIUrl":"10.1183/16000617.0080-2024","url":null,"abstract":"<p><p>Interstitial lung disease (ILD) is a clinical term that refers to a diverse group of non-neoplastic lung diseases. This group includes idiopathic and secondary pulmonary entities that are often associated with progressive pulmonary fibrosis. Currently, therapeutic approaches based on specific structural targeting of pulmonary fibrosis are limited to nintedanib and pirfenidone, which can only slow down disease progression leading to a lower mortality rate. Lung transplantation is currently the only available curative treatment, but it is associated with high perioperative mortality. The pulmonary vasculature plays a central role in physiological lung function, and vascular remodelling is considered a hallmark of the initiation and progression of pulmonary fibrosis. Different patterns of pulmonary fibrosis commonly exhibit detectable pathological features such as morphomolecular changes, including intussusceptive and sprouting angiogenesis, vascular morphometry, broncho-systemic anastomoses, and aberrant angiogenesis-related gene expression patterns. Dynamic cellular interactions within the fibrovascular interface, such as endothelial activation and endothelial-mesenchymal transition, are also observed. This review aims to summarise the current clinical, radiological and pathological diagnostic algorithm for different ILDs, including usual interstitial pneumonia/idiopathic pulmonary fibrosis, non-specific interstitial pneumonia, alveolar fibroelastosis/pleuroparenchymal fibroelastosis, hypersensitivity pneumonitis, systemic sclerosis-related ILD and coronavirus disease 2019 injury. It emphasises an interdisciplinary clinicopathological perspective. Additionally, the review covers current therapeutic strategies and knowledge about associated vascular abnormalities.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"34 175","pages":""},"PeriodicalIF":9.0,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11795288/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143254990","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}