European Respiratory Review最新文献

{"title":"The burden of autoimmune pulmonary alveolar proteinosis: a systematic review.","authors":"Cormac McCarthy, Francesco Bonella, Tisha Wang, Yoshikazu Inoue, Brian Robinson, Bruce C Trapnell","doi":"10.1183/16000617.0237-2025","DOIUrl":"10.1183/16000617.0237-2025","url":null,"abstract":"<p><strong>Background: </strong>Autoimmune pulmonary alveolar proteinosis (aPAP) is characterised by abnormal alveolar surfactant accumulation and reduced pulmonary gas transfer. Disease severity and progression depend on pulmonary surfactant accumulation, the rate of which varies widely among patients. Currently, whole-lung lavage (WLL) is the most widely accepted therapy. This review addresses the burden of aPAP on patients, caregivers and society.</p><p><strong>Methods: </strong>MEDLINE and Embase databases were systematically searched for reports on the manifestations, treatment burden, caregiver impact and healthcare costs of aPAP published after 2000.</p><p><strong>Results: </strong>Out of 1023 publications identified, 50 reported relevant data (for 2855 aPAP patients), including 43 observational studies and seven phase 2/3 trials. Commonly reported symptoms included dyspnoea, cough and sputum production. Clinical manifestations included progressive hypoxaemia, reduced exercise capacity, reduced quality of life, and an increased rate of serious infections. Low prevalence and nonspecific signs and symptoms contributed to delayed diagnosis of aPAP, frequent misdiagnoses, use of multiple tests with nondiagnostic results, and therapies that were inappropriate or exacerbated the disease. WLL was the most frequently administered therapy, and many patients required repeat procedures. Medical care costs were higher for PAP patients than for non-PAP control patients.</p><p><strong>Conclusions: </strong>The results highlight the multifactorial and substantial burden of aPAP on patients. Significant unmet needs remain, particularly in achieving timely and accurate diagnosis and in providing effective, well-tolerated therapies that address the underlying pathophysiology of the disease.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 180","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13058740/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147638242","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":"Biomarker tests of progression from tuberculosis infection to disease: a systematic review.","authors":"Kylie Tingley, Angela Li, Emily L MacLean, Becky Skidmore, Nawal Maredia, Rajalakshmi Ravikumar, Gonzalo G Alvarez, Alberto Matteelli, Giorgia Sulis","doi":"10.1183/16000617.0151-2025","DOIUrl":"10.1183/16000617.0151-2025","url":null,"abstract":"<p><strong>Background: </strong>Tuberculosis (TB) remains a global health threat, with millions of new infections annually. While most infected individuals remain asymptomatic, a subset progresses to active disease. Effective tests predicting TB progression are urgently needed to enhance prevention strategies and reduce the global TB burden.</p><p><strong>Objective: </strong>To evaluate biomarker-based tests designed to predict progression from TB infection to active disease.</p><p><strong>Methods: </strong>We conducted a systematic literature search of four electronic databases for studies published between 2016, when the first trial evaluating biomarker-based tests for TB progression was published, and 2024. Quality assessment was performed using QUADAS-2 (Quality Assessment of Diagnostic Accuracy Studies 2). Data on study design, population, biomarkers, and diagnostic accuracy were extracted and synthesised narratively.</p><p><strong>Results: </strong>19 studies were included, reporting 70 biomarker-based tests for predicting TB progression. These included 23 gene signatures, 14 protein signatures and 33 cytokines/chemokines. Heterogeneity in study populations, methodologies and outcome definitions precluded direct comparisons. Many studies lacked transparency in reporting key population characteristics, reference standards and diagnostic accuracy outcomes, limiting clinical applicability. Most tests demonstrated only moderate predictive accuracy, with no single biomarker approach emerging as a definitive tool for clinical use.</p><p><strong>Conclusions: </strong>No biomarker-based test to predict TB progression is currently ready for clinical implementation. Standardised methodologies, larger validation studies and improved reporting transparency are necessary to advance TB diagnostics and improve early detection efforts. Future research should prioritise refining tests for TB progression by improving their predictive accuracy, identifying appropriate target populations and evaluating their cost-effectiveness and feasibility for integration into TB prevention programmes, particularly in high-burden settings.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13014283/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147510004","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":"Progressive pulmonary fibrosis: the importance of identification and intervention.","authors":"Philip L Molyneaux, Toby M Maher","doi":"10.1183/16000617.0051-2025","DOIUrl":"10.1183/16000617.0051-2025","url":null,"abstract":"<p><p>The concept of progressive pulmonary fibrosis (PPF) was developed to facilitate the identification of patients with an interstitial lung disease (ILD) that is worsening and requires treatment. Various criteria have been proposed to identify PPF, generally based on a deterioration in forced vital capacity alone or with worsening of respiratory symptoms and/or radiological abnormalities. All these criteria are imperfect and based on a limited evidence base. PPF, however it is defined, is associated with high morbidity and mortality. In clinical practice, flexibility is needed in defining ILD progression given differences in the frequencies and methodologies used to monitor patients' disease. Prompt identification of PPF is important to enable timely initiation or escalation of treatment to slow progression of lung fibrosis, consider eligibility for lung transplantation and provide supportive care as needed. In future, earlier treatment of patients at risk of progression may be possible to improve outcomes for patients.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13014284/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147510468","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":"Correspondence regarding: Lung organoids: a new frontier in neonatology and paediatric respiratory medicine.","authors":"Pragnya Das, Vineet Bhandari","doi":"10.1183/16000617.0229-2025","DOIUrl":"10.1183/16000617.0229-2025","url":null,"abstract":"","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13014282/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147510353","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":"Parenteral treprostinil in paediatric pulmonary arterial hypertension: a systematic review and meta-analysis.","authors":"Julie Wacker, Raphael Joye, Maurice Beghetti","doi":"10.1183/16000617.0033-2025","DOIUrl":"10.1183/16000617.0033-2025","url":null,"abstract":"<p><strong>Background: </strong>Paediatric pulmonary arterial hypertension (PAH) shares commonalities with adult disease but is essentially different regarding complexity and is usually more challenging to treat. Current treatment recommendations are based on expert opinion, small-scale paediatric studies and knowledge and consolidated guidelines for adults. Parenteral prostacyclins are recommended for high-risk patients but evidence is limited to cohort studies and retrospective data evaluations. The aim of this article was to summarise the available evidence on the efficacy and safety of parenteral treprostinil for paediatric PAH through a systematic review and to evaluate selected efficacy end-points through meta-analysis.</p><p><strong>Method: </strong>A systematic literature search (January 2000-April 2024) was conducted in PubMed, Google Scholar and clinical trial registries. Eligible studies included those reporting long-term outcomes of parenteral treprostinil in children with PAH. Moreover, a meta-analysis of selected efficacy end-points was performed based on published results from studies meeting predefined criteria.</p><p><strong>Results: </strong>32 studies encompassing 766 paediatric PAH patients treated with parenteral prostacyclins were identified; 649 patients received treprostinil. The meta-analysis was based on five publications including a total of 143 treprostinil-naïve patients. Despite the lack of randomised controlled trials, available data clearly indicate a treatment benefit of parenteral treprostinil in paediatric PAH. Literature data are supported by statistically significant results in the meta-analysis for PAH-relevant efficacy end-points.</p><p><strong>Conclusion: </strong>Based on currently available published data, parenteral treprostinil is effective and safe in the treatment of paediatric PAH.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13014287/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147510345","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":"Reply: Lung organoids: a new frontier in neonatology and paediatric respiratory medicine.","authors":"Lorenzo Zanetto, Luca Bonadies, Raquel Moll-Diaz, Jeffrey Beekman, Maurizio Muraca, Michela Pozzobon, Eugenio Baraldi","doi":"10.1183/16000617.0009-2026","DOIUrl":"10.1183/16000617.0009-2026","url":null,"abstract":"","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13014286/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147510476","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":"Targeting the epithelium in pulmonary fibrosis.","authors":"Muriel Lizé, Christoph H Mayr, Marina Bleck, Lutz Wollin, Christian T Wohnhaas, Irina Shalashova, Heiko Olbrich, Ralf Heilker, Anna Engler, Marie-Therese Bammert, Holger Schlüter, Kerstin Geillinger-Kästle, Devona Soetopo, Stephan Klee, Felix Wolf, Jason J Gokey, Heiko Stahl, Alexander C Klimowicz, A Scott McCall, Jonathan A Kropski, Timothy S Blackwell, Matthew J Thomas","doi":"10.1183/16000617.0225-2025","DOIUrl":"10.1183/16000617.0225-2025","url":null,"abstract":"<p><p>Idiopathic pulmonary fibrosis (IPF) is characterised by progressive scarring primarily of the distal regions of the lung in a distinctive histological pattern. Consequently, drug discovery has focused on the activity of scar-forming fibroblasts, with some success shown by the pharmaceutical agents nintedanib and pirfenidone, and more recently nerandomilast. Yet an unmet medical need remains, pointing toward pathobiology not addressed by standard-of-care therapies. In recent years, single-cell sequencing of patient lung tissue has revealed several novel disease-associated epithelial populations. In addition, we are now beginning to understand, through functional co-culture studies, the importance of cellular crosstalk in both lung homeostasis and disease. Here, we review the advances made in \"omic\" technologies, the approaching spatial profiling revolution, current and future functional cell systems, novel <i>in vivo</i> models, how we assign disease-relevance to test systems <i>via</i> computational gene set enrichment analysis, and link all these elements to clinically relevant biomarkers. We show how epithelial populations, identified and validated through cutting-edge technologies, may provide targets for the next wave of therapeutics for patients with IPF.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13014285/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147510545","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":"Definitions of early COPD and predictors for disease progression: a systematic review.","authors":"Alastair Watson, Ross Davidson, Fu Chuen Kon, Arnav Sharma, Gbenga Adesoye, Bryan Chang, Kane Alexander, Mosea Song, Isobel Soper, Akhilesh Jha, Marie Fisk","doi":"10.1183/16000617.0182-2025","DOIUrl":"10.1183/16000617.0182-2025","url":null,"abstract":"<p><strong>Introduction: </strong>Early chronic obstructive pulmonary disease (COPD) is considered to represent the initial phase of the disease. However, inconsistent terminology and lack of standardised definitions hinders research and clinical application. This systematic review examined clinical research on early COPD, analysed terms and definitions used, and evaluated predictors of disease progression. This serves as a platform to reach consensus and direct future research to target early disease states and improve patient outcomes.</p><p><strong>Methods: </strong>Utilising a standardised protocol, we systematically screened all clinical studies on early COPD. Titles and abstracts were reviewed and compared against inclusion and exclusion criteria. Stage 1 assessed terminology and definitions and stage 2 evaluated predictors of progression. Two independent people reviewed studies at each stage. Study quality was appraised using a modified Downs and Black checklist.</p><p><strong>Results: </strong>We identified 4871 articles, 1759 were screened after duplicate removal. The terms used included PRISm (preserved ratio impaired spirometry) (104 articles), GOLD 0 (Global Initiative for Chronic Obstructive Lung Disease stage 0) (63), early COPD (37), at-risk COPD (35) and pre-COPD (30). Definitions were heterogeneous and proposed early COPD definitions were not routinely used. Stage 2 included 43 full-text articles from cohort studies, of which 93% were of good quality. Predictors of progression included age (n=13 articles), smoking history (12), symptoms (12), exacerbations (one), lung function measures (20), computed tomography metrics (14), risk tools (three) and machine learning approaches (three).</p><p><strong>Conclusion: </strong>We demonstrate an urgent need for consensus on clinically applicable definitions of the early disease course of COPD, prior to diagnosis. We highlight predictors of progression; these need validation to enable stratification of individuals early in their disease trajectory for targeted management to halt or modify progression.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12976884/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147431830","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":"Immunosenescence and susceptibility to respiratory viruses: a state-of-the-art review.","authors":"Paraskevi C Fragkou, Chrysanthi Skevaki, Charalampos D Moschopoulos, Şiran Keske, Hannah Wozniak, Astrid Malézieux-Picard, Virginie Prendki, Jordi Rello","doi":"10.1183/16000617.0248-2025","DOIUrl":"10.1183/16000617.0248-2025","url":null,"abstract":"<p><p>The global rise in the older population poses novel challenges in healthcare systems. Ageing is associated with immunosenescence, a progressive decline and remodelling of the immune system, and with inflammageing, a chronic, low-grade inflammatory process. Both states are associated with increased susceptibility to infections and adverse outcomes, especially in the context of infections. In this review, we examine the molecular and cellular pathophysiological mechanisms of immunosenescence and inflammageing that predispose older adults to increased morbidity and mortality from respiratory viral infections. We also outline the clinical implications of the ageing immune system, along with the most up-to-date evidence on possible biomarkers, preventative measures and treatment options aimed at mitigating the effects of immunosenescence on the vulnerability of older adults in respiratory viral infections.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12976883/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147431878","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":"Pulmonary hypertension associated with nonparenchymal restrictive lung diseases.","authors":"Etienne-Marie Jutant, Marc Humbert, David Montani, Johad Khoury, Yochai Adir","doi":"10.1183/16000617.0187-2025","DOIUrl":"10.1183/16000617.0187-2025","url":null,"abstract":"<p><p>Prior to 2024, pulmonary hypertension (PH) associated with chronic lung diseases (group 3 in the classification of PH) was subclassified according to the type of ventilatory disorder (obstructive, restrictive lung disease or mixed patterns) and not according to the lung disease itself. In 2024, the 7th World Symposium on PH proposed a revised classification, describing associations with specific lung diseases, such as COPD, interstitial lung disease and combined pulmonary fibrosis and emphysema. This update highlights the distinct pathophysiological mechanisms, clinical manifestations, outcomes and management strategies across these subgroups. Of note, a nonparenchymal restrictive lung disease subgroup has been identified, which includes patients with hypoventilation syndromes (due to kyphoscoliosis, diaphragmatic diseases, obesity hypoventilation syndrome, central hypoventilation syndromes, <i>etc.</i>) or pneumonectomy. Paradoxically, despite being one of the earliest forms of PH described, PH associated with nonparenchymal restrictive lung diseases remains among the least studied subtypes. As with all causes of group 3 PH, the primary focus of management is to address the underlying condition whenever possible. There are few data on the efficacy, safety and tolerability of the treatments approved for pulmonary arterial hypertension in this specific population. This review aims to provide updates on this condition and its management, to highlight the mechanisms of PH in each nonparenchymal restrictive lung disease and to study the relevance of the new classification and the necessary avenues of research.</p>","PeriodicalId":12166,"journal":{"name":"European Respiratory Review","volume":"35 179","pages":""},"PeriodicalIF":10.4,"publicationDate":"2026-03-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12976881/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147431891","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}