Respiratory Research最新文献

{"title":"NTHi killing activity is reduced in COPD patients and is associated with a differential microbiome.","authors":"Vancheswaran Gopalakrishnan, Ben Sparklin, Jung Hwan Kim, Jerome Bouquet, Margaret Kehl, Tara Kenny, Christopher Morehouse, Carolina Caceres, Paul Warrener, Ventzislava A Hristova, Susan Wilson, Harini Shandilya, Arnita Barnes, Alexey Ruzin, Junmin Wang, Lisa Oberg, Bastian Angermann, Christopher McCrae, Adam Platt, Daniel Muthas, Sonja Hess, Christine Tkaczyk, Bret R Sellman, Kristoffer Ostridge, Maria G Belvisi, Tom M A Wilkinson, Karl J Staples, Antonio DiGiandomenico","doi":"10.1186/s12931-025-03113-z","DOIUrl":"10.1186/s12931-025-03113-z","url":null,"abstract":"<p><p>Chronic obstructive pulmonary disease (COPD) is a chronic lung disease characterized by airway obstruction and inflammation. Non-typeable Haemophilus influenzae (NTHi) lung infections are common in COPD, promoting frequent exacerbations and accelerated lung function decline. The relationship with immune responses and NTHi are poorly understood. Herein, we comprehensively characterized the respiratory microbiome and mycobiome of patients while investigating microbial dynamics and host immune changes attributable to NTHi killing activity. Mild-to-moderate COPD patients encompassing frequent and infrequent exacerbators and healthy volunteers (HV) were enrolled. Microbial composition, proteomics and NTHi killing activity was analyzed using bronchoalveolar lavage fluid (BALF). In addition, antigen-antibody titers in sera to COPD pathogens were determined using a multiplex assay. Differential abundance analysis revealed an enrichment of Actinobacteria and Bacteroidetes in the BALF of COPD and HV subjects respectively. Significant differences in the IgA titer response were observed against NTHi antigens in COPD vs. HV. Notably, there was also significantly greater killing activity against NTHi in BALF from COPD vs. HV subjects (OR = 5.64; 95% CI = 1.75-20.20; p = 0.001). Stratification of COPD patients by NTHi killing activity identified unique microbial and protein signatures wherein Firmicutes, Actinobacteria and haptoglobin were enriched in patients with killing activity. We report that differences in host immune responses and NTHi-killing activity are associated with microbiome changes in mild-to-moderate COPD. This is suggestive of a potential link between the respiratory microbiome and immune activity against NTHi in the context of COPD pathogenesis even at this disease stage.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"45"},"PeriodicalIF":5.8,"publicationDate":"2025-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11781068/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143069118","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-IL-5 treatment, but not neutrophil interference, attenuates inflammation in a mixed granulocytic asthma mouse model, elicited by air pollution.","authors":"Joyceline De Volder, Annelies Bontinck, Valerie Haelterman, Louis Boon, Guy F Joos, Guy G Brusselle, Tania Maes","doi":"10.1186/s12931-024-03082-9","DOIUrl":"10.1186/s12931-024-03082-9","url":null,"abstract":"<p><strong>Introduction: </strong>Diesel exhaust particles (DEP) have been proven to aggravate asthma pathogenesis. We previously demonstrated that concurrent exposure to house dust mite (HDM) and DEP in mice increases both eosinophils and neutrophils in bronchoalveolar lavage fluid (BALF) and also results in higher levels of neutrophil-recruiting chemokines and neutrophil extracellular trap (NET) formation compared to sole HDM, sole DEP or saline exposure. We aimed to evaluate whether treatment with anti-IL-5 can alleviate the asthmatic features in this mixed granulocytic asthma model. Moreover, we aimed to unravel whether neutrophils modulate the DEP-aggravated eosinophilic airway inflammation.</p><p><strong>Material and methods: </strong>Female C57BL6/J mice were intranasally exposed to saline or HDM and DEP for 3 weeks (subacute model). Interference with eosinophils was performed by intraperitoneal administration of anti-IL-5 (TRFK5), which neutralizes IL-5. Interference with neutrophils and neutrophil elastase was performed by intraperitoneal anti-Ly6G and sivelestat administration, respectively. Outcome parameters included eosinophils subsets (homeostatic EOS and inflammatory EOS), proinflammatory cytokines, goblet cell hyperplasia and airway hyperresponsiveness.</p><p><strong>Results: </strong>The administration of anti-IL-5 significantly decreased eosinophilic responses, affecting both inflammatory and homeostatic eosinophil subsets, upon subacute HDM + DEP exposure while BAL neutrophils, NET formation and other asthma features remained present. Neutrophils were significantly reduced after anti-Ly6G administration in BALF, lung and blood without affecting the eosinophilic inflammation upon HDM + DEP exposure. Sivelestat treatment tended to decrease BALF inflammation, including eosinophils, upon HDM + DEP exposure, but did not affect lung inflammation.</p><p><strong>Conclusion: </strong>Inhibition of IL-5 signalling, but not neutrophil interventions, significantly attenuates eosinophilic inflammation in a mouse model of mixed granulocytic asthma, elicited by air pollution exposure.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"43"},"PeriodicalIF":5.8,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11773929/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061269","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Fibrosis in PCLS: comparing TGF-β and fibrotic cocktail.","authors":"Carlos Machahua, Thomas M Marti, Patrick Dorn, Manuela Funke-Chambour","doi":"10.1186/s12931-025-03110-2","DOIUrl":"10.1186/s12931-025-03110-2","url":null,"abstract":"<p><strong>Introduction: </strong>Fibrotic cocktail (FC) is a combination of pro-fibrotic and pro-inflammatory mediators that induces early fibrotic changes in organotypic lung models. We hypothesised that transforming growth factor beta 1 (TGF-β1) alone induces a pro-fibrotic effect similar to FC. Our aim was to compare the pro-fibrotic effects of TGF-β1 with FC in human precision-cut lung slices (PCLS).</p><p><strong>Methods: </strong>PCLS from \"healthy\" lung tissue of cancer patients undergoing surgery (n = 7) were incubated with TGF-β1, FC or control for 72 h. Gene expression markers for myofibroblasts differentiation, extracellular matrix (ECM), as well as TGF-β receptors were assessed (RT-qPCR). ECM proteins expression in lysates and supernatant was assessed by ELISA and immunofluorescence.</p><p><strong>Results: </strong>We found that TGF-β1 significantly increased gene expression of ACTA2, COL1A1, CCN2, and VIM compared to control but also compared to FC. FC showed a significant increase of matrix metalloproteinase (MMP) 7 and 1 compared to control, while TGF-β receptor 2 was lower after FC compared to TGF-β1 or control. FC or TGF-β1 showed similar fibronectin protein expression in lysates and supernatants, while type I collagen protein expression in lysates was significantly greater with TGF-β1 compared to control.</p><p><strong>Conclusions: </strong>Our findings show that TGF-β1 induces consistent pro-fibrotic changes in PCLS after 72 h. Compared to TGF-β1, FC treatment resulted in reduced gene expression of TGF-β receptor 2 and increased MMPs expression, potentially mitigating the early pro-fibrotic effects. Selecting specific pro-fibrotic stimuli may be preferable depending on the research question and time point of interest in lung fibrosis studies using PCLS.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"44"},"PeriodicalIF":5.8,"publicationDate":"2025-01-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11776118/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143061270","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Correction: Precision cut lung slices: an integrated ex vivo model for studying lung physiology, pharmacology, disease pathogenesis and drug discovery.","authors":"Cynthia Koziol-White, Eric Gebski, Gaoyaun Cao, Reynold A Panettieri","doi":"10.1186/s12931-024-03085-6","DOIUrl":"10.1186/s12931-024-03085-6","url":null,"abstract":"","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"42"},"PeriodicalIF":5.8,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11773737/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143053943","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Comparative analysis of ambient, in-home, and personal exposures reveals associations between breathing zone pollutant levels and asthma exacerbations in high-risk children.","authors":"Camille M Moore, Jonathan Thornburg, Elizabeth A Secor, Katharine L Hamlington, Allison M Schiltz, Kristy L Freeman, Jamie L Everman, Tasha E Fingerlin, Andrew H Liu, Max A Seibold","doi":"10.1186/s12931-025-03114-y","DOIUrl":"10.1186/s12931-025-03114-y","url":null,"abstract":"<p><strong>Background: </strong>Air pollution is associated with poor asthma outcomes in children. However, most studies focus on ambient or indoor monitor pollution levels. Few studies evaluate breathing zone exposures, which may be more consequential for asthma outcomes.</p><p><strong>Methods: </strong>We measured personal exposures to NO₂, O₃, PM₁₀ and PM₁₀ constituents, including black carbon (BC), brown carbon (BrC), environmental tobacco smoke (ETS), endotoxins, and 𝛽-glucan, in a cohort of children with exacerbation-prone asthma for 72 h using wearable monitors. Personal exposures were compared to concentrations from in-home monitors in the child's bedroom and ambient EPA air quality monitoring using correlation analyses. Personal exposures were tested for association with lung function and compared between participants with and without well-controlled asthma and signs of exacerbation in the prior 60 days using censored regression with robust standard errors.</p><p><strong>Results: </strong>81 children completed 219 monitoring sessions. Personal NO₂, O₃, and PM₁₀ exposures ranged from < 2 to 99.1 parts per billion (ppb), < 1.5 to 23.3 ppb, and < 1 to 141.9 𝜇g/m³, respectively. Personal endotoxin ranged from 0.04 to 101.3 EU/m³, 𝛽-glucan from 18.5 to 1,162 pg/m³, BC from < 0.3 to 46.9 𝜇g/m³, BrC from < 0.3 to 6.1 𝜇g/m³, and ETS from < 0.3 to 56.6 𝜇g/m³. Correlations between personal and ambient PM₁₀, NO₂, and O₃ concentrations were poor, whereas personal PM₁₀ and NO₂ correlated with in-home concentrations. In-home monitoring less frequently detected BrC (Personal:79% > lower limit of detection, Home:36.8%) and ETS (Personal:83.7%, Home:4.1%) than personal exposures, and detected BC in participants without personal exposure (Personal: 26.5%, Home: 96%). Personal exposures were not significantly associated with lung function or daily asthma control. Children requiring corticosteroid treatment for asthma exacerbation within 60 days of exposure monitoring had 1.98, 2.21 and 2.04 times higher personal exposures to BrC (p < 0.001; 95% CI: 1.43-2.37), ETS (p = 0.007; 95% CI: 1.25-3.91), and endotoxin (p = 0.012; 95% CI: 1.14-3.68), respectively.</p><p><strong>Conclusions: </strong>Although in-home monitoring was correlated with personal exposure to PM₁₀ and NO₂, in-home detection of ETS and BrC was not associated with personal exposures. Personal PM₁₀ exposures in general, as well as BrC, ETS, and endotoxin levels were associated with recent childhood asthma exacerbations.</p><p><strong>Clinical trial number: </strong>Not applicable.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"40"},"PeriodicalIF":5.8,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11773965/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143053940","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"HMOX1 as a potential drug target for upper and lower airway diseases: insights from multi-omics analysis.","authors":"Enhao Wang, Shazhou Li, Yang Li, Tao Zhou","doi":"10.1186/s12931-025-03124-w","DOIUrl":"10.1186/s12931-025-03124-w","url":null,"abstract":"<p><strong>Background: </strong>Oxidative stress is key in inflammatory airway diseases. Heme oxygenase 1 (HMOX1) regulates oxidative stress, but its role in airway diseases needs exploration.</p><p><strong>Methods: </strong>Differentially expressed genes (DEGs) between healthy nasal mucosa and chronic rhinosinusitis with nasal polyps (CRSwNP) were identified from Gene Expression Omnibus (GEO). Candidate genes were further screened using Gene Set Enrichment Analysis (GSEA) and Random Forest (RF) algorithms. Causal inference between candidate genes and upper and lower airway diseases (CRSwNP, allergic rhinitis (AR), and asthma (AS)) was conducted using bidirectional two-sample Mendelian randomization (TwoSampleMR) analysis. Single-cell RNA sequencing (scRNA-seq) data were used to determine the cellular localization and intercellular interactions of candidate genes. Molecular docking was used to identify potential therapeutic agents.</p><p><strong>Results: </strong>HMOX1 expression was significantly elevated in CRSwNP. TwoSampleMR analysis indicated a negative causal relationship between HMOX1 exposure and the occurrence of upper and lower airway diseases (CRSwNP [(odds ratio (OR)/95% confidence interval (CI): 0.945/(0.893-0.999), P = 0.044], AR [OR/95% CI: 0.997/(0.994-0.999), P = 0.007], and AS [OR/95% CI: 0.935/(0.895-0.977), P = 0.003]). scRNA-seq data revealed HMOX1 localization in M2 macrophages. Molecular docking identified 15 antioxidants, including Acetylcysteine and Quercetin, that can upregulate HMOX1 expression.</p><p><strong>Conclusion: </strong>HMOX1 may have a protective role in the pathogenesis of upper and lower airway diseases (CRSwNP, AR, and AS) by modulating oxidative stress. Antioxidants that increase HMOX1 expression could offer new therapeutic avenues for these diseases.</p><p><strong>Clinical trial: </strong>Not applicable.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"41"},"PeriodicalIF":5.8,"publicationDate":"2025-01-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11773792/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143053944","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Peripheral blood miRNAs are associated with airflow below threshold in children with asthma.","authors":"Anshul Tiwari, Brian D Hobbs, Rinku Sharma, Jiang Li, Alvin T Kho, Sami Amr, Juan C Celedón, Scott T Weiss, Craig P Hersh, Kelan G Tantisira, Michael J McGeachie","doi":"10.1186/s12931-025-03116-w","DOIUrl":"10.1186/s12931-025-03116-w","url":null,"abstract":"<p><strong>Background: </strong>MicroRNAs (miRNAs) are crucial post-transcriptional regulators involved in inflammatory diseases, such as asthma. Poor lung function and airflow issues in childhood are linked to the development of chronic obstructive pulmonary disease (COPD) in adulthood.</p><p><strong>Methods: </strong>We analyzed small RNA-Seq data from 365 peripheral whole blood samples from the Genetics of Asthma in Costa Rica Study (GACRS) for association with airflow levels measured by FEV1/FVC. Differentially expressed (DE) miRNAs were identified using DESeq2 in R, adjusting for covariates and applying a 10% false discovery rate (FDR). The analysis included 361 samples and 649 miRNAs. The two DE miRNAs were further tested for association with airflow obstruction in a study of adult former smokers with and without COPD.</p><p><strong>Results: </strong>We found 1 upregulated and 1 downregulated miRNA in participants with airflow below the threshold compared to those above it. In the adult study, the same miRNAs were upregulated and downregulated in individuals with FEV1/FVC < 0.7 versus those with FEV1/FVC > 0.7, showing suggestive statistical evidence. The target genes of these miRNAs were enriched for PI3K-Akt, Hippo, WNT, MAPK, and focal adhesion pathways.</p><p><strong>Conclusions: </strong>Two differentially expressed miRNAs were associated with airflow levels in children with asthma and airflow obstruction in adults with COPD. This suggests that shared genetic regulatory systems may influence childhood airflow and contribute to adulthood airflow obstruction.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"38"},"PeriodicalIF":5.8,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11763123/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143041923","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Single, double, and triple-hit strategies to establish a long-term premature rabbit model of bronchopulmonary dysplasia.","authors":"Chiara Catozzi, Francesca Stretti, Enrica Scalera, Matteo Storti, Angelo Modena, Giorgio Aquila, Gino Villetti, Erica Ferrini, Andrea Grandi, Franco Fabio Stellari, Francesca Ravanetti, Luisa Ragionieri, Roberta Ciccimarra, Matteo Zoboli, Christina Brandenberger, Henri Schulte, Xabier Murgia, Maurizio Civelli, Francesca Ricci","doi":"10.1186/s12931-024-03053-0","DOIUrl":"10.1186/s12931-024-03053-0","url":null,"abstract":"<p><strong>Background: </strong>Bronchopulmonary dysplasia (BPD) is a chronic lung condition of premature neonates, yet without an established pharmacological treatment. The BPD rabbit model exposed to 95% oxygen has been used in recent years for drug testing. However, the toxicity of the strong hyperoxic hit precludes a longer-term follow-up due to high mortality after the first week of life. This study aimed to extend the preterm rabbit model to postnatal day (PND) 14 to mimic the evolving phase of BPD and enable the investigation of therapeutic interventions at later and more relevant time points.</p><p><strong>Methods: </strong>Preterm rabbit pups delivered on the 28th day of gestation were either exposed to room air or different degrees of hyperoxia (50% and 70% O₂) for 14 days. Single (immediately after birth) or double (at birth and at PND5) intratracheal lipopolysaccharide (LPS) administrations were also tested in combination with 50% O₂. Age-matched rabbits delivered vaginally at term were used as controls. Survival, weight gain, lung function, pulmonary artery micro-ultrasound Doppler analysis, lung histology (alveolarization, lung injury score, and design-based stereology), and longitudinal micro-CT imaging were used to compare the outcomes at PND14.</p><p><strong>Results: </strong>Premature birth itself, without any other BPD hit, was associated with lung function deficits, delayed lung development, and cardiovascular abnormalities. The BPD-like lung phenotype was enhanced by 70% O₂ but not by 50% O₂ hyperoxia. Intratracheal LPS delivered immediately after birth was associated with significantly higher lung injury scores at PND14 and increased tissue damping, a marker of parenchymal air resistance.</p><p><strong>Conclusion: </strong>Several strategies are feasible to extend the preterm rabbit model of BPD to PND14. Preterm birth at the saccular phase itself, even in the absence of other postnatal BPD hits, was associated with lung function deficits, delayed lung development, and cardiovascular abnormalities compared with age-matched term rabbit pups. Enhanced BPD-like phenotypes can be further achieved by continued exposure to moderate hyperoxia (70% O₂) and the intratracheal administration of LPS.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"35"},"PeriodicalIF":5.8,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11762538/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143042318","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Automated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients.","authors":"Julien Guiot, Monique Henket, Fanny Gester, Béatrice André, Benoit Ernst, Anne-Noelle Frix, Dirk Smeets, Simon Van Eyndhoven, Katerina Antoniou, Lennart Conemans, Janine Gote-Schniering, Hans Slabbynck, Michael Kreuter, Jacobo Sellares, Ioannis Tomos, Guang Yang, Clio Ribbens, Renaud Louis, Vincent Cottin, Sara Tomassetti, Vanessa Smith, Simon L F Walsh","doi":"10.1186/s12931-025-03117-9","DOIUrl":"10.1186/s12931-025-03117-9","url":null,"abstract":"<p><strong>Background: </strong>Systemic sclerosis (SSc) is a rare connective tissue disease associated with rapidly evolving interstitial lung disease (ILD), driving its mortality. Specific imaging-based biomarkers associated with the evolution of lung disease are needed to help predict and quantify ILD.</p><p><strong>Methods: </strong>We evaluated the potential of an automated ILD quantification system (icolung^®) from chest CT scans, to help in quantification and prediction of ILD progression in SSc-ILD. We used a retrospective cohort of 75 SSc-ILD patients to evaluate the potential of the AI-based quantification tool and to correlate image-based quantification with pulmonary function tests and their evolution over time.</p><p><strong>Results: </strong>We evaluated a group of 75 patients suffering from SSc-ILD, either limited or diffuse, of whom 30 presented progressive pulmonary fibrosis (PPF). The patients presenting PPF exhibited more extensive lesions (in % of total lung volume (TLV)) based on image analysis than those without PPF: 3.93 (0.36-8.12)* vs. 0.59 (0.09-3.53) respectively, whereas pulmonary functional test showed a reduction in Force Vital Capacity (FVC)(pred%) in patients with PPF compared to the others : 77 ± 20% vs. 87 ± 19% (p < 0.05). Modifications of FVC and diffusing capacity of the lungs for carbon monoxide (DLCO) over time were correlated with longitudinal radiological ILD modifications (r=-0.40, p < 0.01; r=-0.40, p < 0.01 respectively).</p><p><strong>Conclusion: </strong>AI-based automatic quantification of lesions from chest-CT images in SSc-ILD is correlated with physiological parameters and can help in disease evaluation. Further clinical multicentric validation is necessary in order to confirm its potential in the prediction of patient's outcome and in treatment management.</p>","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"39"},"PeriodicalIF":5.8,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11762107/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143041373","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Risk of lung disease with the Pi*SS genotype of alpha-1 antitrypsin: the evidence in context.","authors":"Helen O'Brien, Cormac McCarthy, Alessandro N Franciosi","doi":"10.1186/s12931-024-03066-9","DOIUrl":"10.1186/s12931-024-03066-9","url":null,"abstract":"","PeriodicalId":49131,"journal":{"name":"Respiratory Research","volume":"26 1","pages":"37"},"PeriodicalIF":5.8,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11763142/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143042225","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}