European Respiratory Review最新文献

{"title":"Ageing-associated regenerative failure in the lung: stem cell senescence and transitional cell persistence in idiopathic pulmonary fibrosis.","authors":"Masahiro Yoshida, Jun Araya","doi":"10.1183/16000617.0278-2025","DOIUrl":"https://doi.org/10.1183/16000617.0278-2025","url":null,"abstract":"<p><p>Alveolar regeneration failure due to alveolar stem cell senescence is a defining feature of idiopathic pulmonary fibrosis (IPF), yet the epithelial mechanisms underlying this dysfunction remain incompletely understood. Recent single-cell and lineage-tracing studies have identified distinct transitional epithelial states, such as pre-alveolar type-1 transitional cells, damage-associated transient progenitors, and alveolar-basal intermediates, which normally serve as intermediates during type 2 alveolar cell (AT2) to type 1 alveolar cell (AT1) differentiation. While these states are transient and successfully resolved during acute lung injury, these populations persist abnormally in IPF lungs, exhibiting features of senescence, cell-cycle arrest, and impaired differentiation.We review recent evidence showing how premature alveolar epithelial senescence, suppression of developmental regenerative programmes, and sustained transforming growth factor-β signalling within the fibrotic niche contribute to this disruption. These factors promote the accumulation of dysfunctional transitional cells, such as cytokeratin (KRT)8⁺/KRT17⁺ basaloid cells, which fail to regenerate alveolar epithelium and instead contribute to fibrosis and bronchiolisation through pathological crosstalk with macrophages and fibroblasts. Notably, similar transitional states appear in COVID-19-associated acute respiratory distress syndrome, but they typically exhibit minimal features of senescence and resolve during regeneration, underscoring the pathological importance of accelerated epithelial senescence in IPF.Understanding the molecular checkpoints that regulate transitional cell fate may offer novel strategies to restore regenerative capacity in fibrosing lung diseases.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13482673/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148790010","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":"Weight loss for obstructive sleep apnoea: first-line therapy should not mean single-line therapy.","authors":"ZhiKang Yin, Liang Wei","doi":"10.1183/16000617.0169-2026","DOIUrl":"https://doi.org/10.1183/16000617.0169-2026","url":null,"abstract":"","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13482737/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148790111","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":"\"Exacerbations of bronchiectasis.\" A. De Angelis, E.D. Johnson, S. Sutharsan, <i>et al</i>. <i>Eur Respir Rev</i> 2024; 33: 240085.","authors":"","doi":"10.1183/16000617.5085-2024","DOIUrl":"https://doi.org/10.1183/16000617.5085-2024","url":null,"abstract":"","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13482739/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148790020","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":"Murine models of pulmonary fibrosis: mechanisms, limitations and translational insights.","authors":"Karen F Lou, McKenzie M Fulcer, Mary J Lotesto, Jennifer M Davis, Tatiana P Ortiz Serrano, Ruihua Ma, Karen M Ridge","doi":"10.1183/16000617.0033-2026","DOIUrl":"https://doi.org/10.1183/16000617.0033-2026","url":null,"abstract":"<p><p>Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, irreversible interstitial lung disease characterised by excessive collagen deposition, aberrant tissue remodelling, and impaired gas exchange, ultimately leading to respiratory failure and death. IPF carries a poor prognosis, with limited therapeutic options; currently approved treatments slow disease progression but do not reverse established fibrosis, and lung transplantation remains the only definitive therapy. Moreover, the prevalence and incidence of IPF continue to rise worldwide. These challenges underscore the urgent need to better understand the pathological mechanisms driving IPF, enabling the development of more effective therapeutic strategies. Robust and appropriate animal models are essential for investigating disease pathogenesis and therapeutic response. Although multiple murine models of pulmonary fibrosis are widely used, no single model fully recapitulates the clinical, pathological and temporal features of human IPF. In this review, we systematically summarise commonly used and new murine models of pulmonary fibrosis, emphasising induction methods, technical requirements, the time course of fibrosis development and persistence of fibrosis. Additionally, we highlight the advantages and limitations of each model, discuss how they inform mechanistic and translational studies, and provide practical guidance for selecting appropriate experimental systems based on specific scientific questions, with the goal of improving rigor and relevance in preclinical pulmonary fibrosis research.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13482731/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148789955","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":"Vaccinations in patients with interstitial lung diseases: a narrative review.","authors":"Giovanni Franco, Umberto Zanini, Giulia Peri, Isabella Galbardi, Samuele Promi, Sofia Maria Mazzotta, Paola Faverio, Raphaël Borie, Fabrizio Luppi, Bruno Crestani","doi":"10.1183/16000617.0079-2026","DOIUrl":"https://doi.org/10.1183/16000617.0079-2026","url":null,"abstract":"<p><p>Interstitial lung diseases (ILDs) comprise a heterogeneous group of lung disorders marked by progressive fibrosis and increased vulnerability to respiratory infections, which can trigger sudden outbreaks and worsen outcomes. Vaccination is a key preventive strategy in ILD patients to reduce infection-related morbidity and mortality. This review examines the importance of immunisation in individuals with ILDs, with particular emphasis on influenza, pneumococcal and severe acute respiratory syndrome coronavirus 2 vaccines endorsed by major health organisations. Despite evidence of their effectiveness, vaccination rates among patients with ILD remain low. Tailored vaccine strategies may enhance protection in this group. Additionally, although data are limited, immunisations against respiratory syncytial virus, <i>Haemophilus influenzae</i>, varicella-zoster virus, and pertussis may provide additional protection, particularly in older adults and those receiving immunosuppressive therapy. Hepatitis B vaccination should also be considered for patients on immunosuppressive therapies to prevent infection. Healthcare providers should actively encourage vaccination and address obstacles to immunisation in ILD patients. More research is necessary to assess the safety, effectiveness and optimal vaccination approaches for this vulnerable group. Ultimately, implementing systematic vaccination programmes and clear clinical guidelines is essential to improve preventive care and health outcomes for those with ILDs.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13482714/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148790126","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":"Patient-reported outcome measures applied in chronic diseases relevant to asthma remission: a scoping review.","authors":"Allison Michaud, John Politis, Lachlan Faktor, Philip G Bardin, Amy H Y Chan, Paul Leong","doi":"10.1183/16000617.0292-2025","DOIUrl":"10.1183/16000617.0292-2025","url":null,"abstract":"<p><strong>Background: </strong>Asthma remission is a meaningful treatment goal that requires patient and healthcare provider agreement. However, there is currently no validated way to incorporate patient perspectives of remission in this process. Patient-reported outcome measures (PROMs) can allow comprehensive assessment of remission by capturing the impact on individual patient experiences. The aim of this study was to identify PROMs used to define remission in other chronic diseases, thereby informing development of a PROM for asthma remission.</p><p><strong>Methods: </strong>A scoping review of primary research studies was conducted using five databases. Eligible studies reported on the development or validation of PROMs that assess remission in chronic diseases. Data were extracted and analysed thematically to identify types of PROMs, health domains assessed and methodological quality.</p><p><strong>Results: </strong>Nine studies met inclusion criteria, covering 12 PROMs for diseases such as rheumatoid arthritis, inflammatory bowel disease and ankylosis spondyloarthritis. Domains most frequently assessed included disease-specific symptoms (n=11), generalised symptoms (n=5) and mental health (n=2). PROMs generally asked patients about their current health (n=8) although some PROMs had longer recall periods (n=4). Only one PROM was developed with direct patient involvement.</p><p><strong>Conclusions: </strong>PROMs currently employed in other chronic diseases signify significant complexity when attempting to capture multidimensional aspects of disease remission from a patient perspective. However, fundamental domains and other health elements can be distinguished that will aid development of robust, patient-informed PROMs that align with patient-centred experiences of remission, thereby improving shared decision-making in asthma.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13439356/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677531","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":"Large animal models of right heart failure due to pulmonary hypertension: a bridge to translational success.","authors":"Sally E Prins, Madelyn J Blake, Kristi L Helke, Thomas Di Salvo, Ryan J Tedford, Steven Hsu, Kurt W Prins","doi":"10.1183/16000617.0015-2026","DOIUrl":"10.1183/16000617.0015-2026","url":null,"abstract":"<p><p>Right heart failure drives morbidity and mortality in pulmonary arterial hypertension (PAH). At present, there are no chronic pharmaceutical interventions that directly augment right heart function in humans despite many promising results in rodent studies. The lack of translational success may be due to the relative scarcity of studies examining large animal models of right heart failure. However, in recent years, a rapid expansion of large animal investigations worldwide is generating new important physiological and mechanistic data. Large animal models provide more human-relevant biology, which can be paired with physiological assessments and multi-omic profiling to expand our knowledge of right heart failure. Here, we highlight findings obtained from pigs, sheep, cows and dogs, and define some potential limitations of each large animal model. In addition, we outline important questions that could be addressed in future research. Although imperfect, large animals will likely serve important roles in the development of right heart-directed therapies ranging from mechanical interventions to pharmaceutical approaches as they move up the translational pipeline. Hopefully, the field will continue these critical lines of research to promote the discovery of novel therapeutics for our patients who are in desperate need of new options.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13439341/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677612","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":"Paediatric obstructive sleep apnoea and early cardiovascular risk phenotypes: an evidence review.","authors":"Ping Wei, Hua Yang, Yi Yang, Yijun Liu, Wei Kou","doi":"10.1183/16000617.0052-2026","DOIUrl":"10.1183/16000617.0052-2026","url":null,"abstract":"<p><p>Adult obstructive sleep apnoea (OSA) is an established cardiovascular risk factor, but paediatric OSA has been framed mainly around neurocognition and growth. We reviewed PubMed, Ovid-Embase and Web of Science (2000-2026), prioritising eligible paediatric studies with quantifiable outcomes (ambulatory/office blood pressure (BP), autonomic indices/heart-rate variability, endothelial function/arterial stiffness, carotid intima-media thickness (cIMT) and echocardiographic structure/function) and key interventional data. Across cohorts, 24-h ambulatory blood pressure monitoring (ABPM) most consistently demonstrates higher nocturnal BP and reduced dipping; emerging exposure metrics such as hypoxic burden (the integrated depth and duration of oxygen desaturation across sleep) may capture physiological stress better than the apnoea-hypopnoea index alone. Autonomic imbalance, including sympathetic predominance, altered sleep-stage autonomic modulation and reduced baroreflex gain, provides a plausible bridge between respiratory events and early vascular/cardiac remodelling, and often improves after adenotonsillectomy. Vascular data suggest early functional abnormalities (endothelial reactivity, wave reflection/central haemodynamics) with smaller and inconsistent differences in structural markers, such as cIMT, strongly modified by obesity and development. Cardiac studies most reproducibly show increased left-ventricular mass/geometry and impaired diastolic relaxation, while right-heart and pulmonary vascular signals appear most relevant in selected higher-risk groups. Treatment effects appear more readily detectable in autonomic/endothelial measures than in office BP over short follow-up, reinforcing ABPM as a preferred research and selected high-risk clinical end-point. Paediatric OSA should therefore be approached through an early-risk framework, including identifying higher-risk phenotypes, quantifying nocturnal haemodynamic burden when feasible and integrating OSA therapy with weight and guideline-directed BP management.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13439383/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677525","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":"Normal limits for the right ventricle-pulmonary circulation exercise echocardiography: a meta-analysis.","authors":"Francesco Ferrara, Valentina Capone, Monica Franzese, Andreina Carbone, Erberto Carluccio, Rosanna Castaldo, Rodolfo Citro, Antonio Cittadini, Anna D'Agostino, Michele D'Alto, Alessandra Maria Esposito, Giovanni Esposito, Luna Gargani, Stefano Ghio, Graziella Lacava, Alberto Maria Marra, Ciro Mauro, Emanuela Passaro, Salvatore Rega, Andrea Soricelli, Ekkehard Grünig, Robert Naeije, Eduardo Bossone","doi":"10.1183/16000617.0188-2025","DOIUrl":"10.1183/16000617.0188-2025","url":null,"abstract":"<p><p>Normal limits for exercise Doppler echocardiography (ex-TTE) measurements of the right ventricle (RV), pulmonary artery (PA) and left atrial (LA) unit remain inadequately defined. This meta-analysis aims to establish normal limits for measurements of the RV-PA-LA unit. A comprehensive literature search of Medline, Web of Science and Scopus (1 January 1999-December 2024) was performed. We included ex-TTE studies performed using a supine/semi-recumbent cycle ergometer in healthy subjects that reported the following parameters: tricuspid regurgitation velocity (TRV), systolic pulmonary artery pressure (sPAP), mean pulmonary artery pressure (mPAP), mPAP/cardiac output (CO) slope, mitral Doppler E to tissue Doppler e' ratio (E/e'), tricuspid annular plane excursion (TAPSE) and TAPSE/sPAP. Summary mean estimates were calculated using a restricted maximum-likelihood random-effects model. Lower and upper limits of normal (LLN and ULN) were defined as the 5th and 95th percentiles. Between-study heterogeneity was assessed using the Q-statistic and quantified with the inconsistency index. Data on 1122 healthy subjects (mean±sd age: 42.8±18.3 years) across 19 eligible studies were analysed. The pooled mean estimates and limits of normal for ex-TTE measurements were as follows: TRV 2.5 m·s^-1 (ULN 3.4), sPAP 38.5 mmHg (ULN 57.8), mPAP 29.8 mmHg (ULN 43.0), mPAP/CO slope 1.4 mmHg·min·L^-1 (ULN 3.0), E/e' 7.1 (ULN 11.5), TAPSE 31.8 mm (LLN 24.6), TAPSE/sPAP 0.8 mm·mmHg^-1 (LLN 0.4). Despite notable heterogeneity for several measurements, the present meta-analysis provides a robust framework for defining normal reference limits for ex-TTE measurements of the RV-PA-LA unit.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13439342/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677528","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":"How experimental models have unlocked hidden mechanisms of hypersensitivity pneumonitis.","authors":"Sandra Cabrera, Annie Pardo, Moisés Selman","doi":"10.1183/16000617.0010-2026","DOIUrl":"10.1183/16000617.0010-2026","url":null,"abstract":"<p><p>Hypersensitivity pneumonitis (HP) is a complex immune-mediated interstitial lung disease triggered by repeated or persistent inhalation of a variety of inhaled antigens in genetically predisposed individuals. The disease encompasses a wide clinical spectrum and may present as predominantly inflammatory or fibrotic forms, the latter associated with irreversible architectural distortion and progressive loss of pulmonary function. Although mechanisms involving dysregulated T-cell activation, aberrant antigen processing and maladaptive tissue-repair pathways are recognised as central contributors to HP pathogenesis, the precise mechanisms and the sequence of immunologic events that occur during inflammation and mainly those that drive the transition from inflammation to fibrosis remains unclear. Animal models, particularly murine systems, have helped in dissecting these mechanisms, yet they continue to fall short in fully recapitulating the heterogeneity and chronicity observed in human HP. In this review, we provide an in-depth and critical examination of existing wild-type and genetically modified mouse models used to investigate HP, with emphasis on antigen sources, exposure paradigms and the strengths and limitations of each approach. We synthesise current insights into how specific genes, signalling pathways and immune cell subsets, including T-helper cell polarisation, regulatory T-cell function, dendritic cell activation and immune complexes contribute to disease initiation, amplification and progression.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 181","pages":""},"PeriodicalIF":10.8,"publicationDate":"2026-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13439357/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148677598","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}