Pub Date : 2026-03-12DOI: 10.1183/13993003.02651-2025
Ganesh Raghu,Sandeep Sahay,Laura C Price,Jason Weatherald,Samuel G Rayner,Jürgen Behr,Athénaïs Boucly,Manisit Das,Vallerie McLaughlin,Franck Rahaghi,Cedric Rutland,Jay H Ryu,Rajan Saggar,Marc Humbert
Pulmonary hypertension (PH) is a common sequela of interstitial lung diseases (ILDs) and is associated with poor prognosis and quality of life. The diagnosis and management of PH associated with ILD (ILD-PH) are challenging, due in part to the heterogeneity of ILD subtypes, difficulty distinguishing symptoms and signs of ILD progression from manifestations of PH, lack of specific biomarkers, and the requirement of invasive right heart catheterisation (RHC) to diagnose and assess the severity of PH. This state-of-the-art review provides a comprehensive overview of the clinical characteristics, pathophysiology, diagnosis, prognosis, and treatment of ILD-PH. It also identifies promising areas for future research, such as the development and validation of novel biomarkers and imaging techniques and further evaluation of the efficacy and safety of pharmacologic therapies for PH in patients with ILD. Given the inherent complexity of diagnosing and managing heterogeneous ILD subtypes, there is a clear need for multidisciplinary and personalised care strategies for ILD-PH. Dedicated attention and further research to improve diagnostic and treatment interventions are warranted to help develop much-needed, evidence-based guidelines and to improve outcomes that are meaningful for patients with ILD-PH.
{"title":"Pulmonary Hypertension Associated With Interstitial Lung Disease: State-of-the-Art Review.","authors":"Ganesh Raghu,Sandeep Sahay,Laura C Price,Jason Weatherald,Samuel G Rayner,Jürgen Behr,Athénaïs Boucly,Manisit Das,Vallerie McLaughlin,Franck Rahaghi,Cedric Rutland,Jay H Ryu,Rajan Saggar,Marc Humbert","doi":"10.1183/13993003.02651-2025","DOIUrl":"https://doi.org/10.1183/13993003.02651-2025","url":null,"abstract":"Pulmonary hypertension (PH) is a common sequela of interstitial lung diseases (ILDs) and is associated with poor prognosis and quality of life. The diagnosis and management of PH associated with ILD (ILD-PH) are challenging, due in part to the heterogeneity of ILD subtypes, difficulty distinguishing symptoms and signs of ILD progression from manifestations of PH, lack of specific biomarkers, and the requirement of invasive right heart catheterisation (RHC) to diagnose and assess the severity of PH. This state-of-the-art review provides a comprehensive overview of the clinical characteristics, pathophysiology, diagnosis, prognosis, and treatment of ILD-PH. It also identifies promising areas for future research, such as the development and validation of novel biomarkers and imaging techniques and further evaluation of the efficacy and safety of pharmacologic therapies for PH in patients with ILD. Given the inherent complexity of diagnosing and managing heterogeneous ILD subtypes, there is a clear need for multidisciplinary and personalised care strategies for ILD-PH. Dedicated attention and further research to improve diagnostic and treatment interventions are warranted to help develop much-needed, evidence-based guidelines and to improve outcomes that are meaningful for patients with ILD-PH.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"28 15 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147439208","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-03-12DOI: 10.1183/13993003.02506-2025
Daniel Vena,Phillip Huyett,Scott A Sands
{"title":"Reply to: More correlates - not predictors - of hypoglossal nerve stimulation effectiveness.","authors":"Daniel Vena,Phillip Huyett,Scott A Sands","doi":"10.1183/13993003.02506-2025","DOIUrl":"https://doi.org/10.1183/13993003.02506-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"8 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147439553","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-03-12Print Date: 2026-03-01DOI: 10.1183/13993003.01760-2025
Andreas Hoheisel, Sebastian Fähndrich, Anna Salina, Eleni Papakonstantinou, Maria Pascarella, Leticia Grize, Daiana Stolz
{"title":"Bronchodilator reversibility, body plethysmographic phenotypes, and mortality in patients with COPD or PRISm.","authors":"Andreas Hoheisel, Sebastian Fähndrich, Anna Salina, Eleni Papakonstantinou, Maria Pascarella, Leticia Grize, Daiana Stolz","doi":"10.1183/13993003.01760-2025","DOIUrl":"10.1183/13993003.01760-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2026-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145932819","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-03-12DOI: 10.1183/13993003.01874-2025
Bernt Van Den Blink,Surinder S Birring,Nesrin Mogulkoc,Sibel N Atis,Rohit Gupta,Julien Guiot,Simon P Hart,Eva M Carmona Porquera,Hajira B Koeller,Natasha Arocho,Chantal M Petit,Jill Denning,Min Lin,Robert P Baughman,Marlies Wijsenbeek-Lourens,Theodore F Reiss,
BACKGROUNDTreatment options for chronic pulmonary sarcoidosis are limited in efficacy and by tolerability concerns. Namilumab, an investigational humanized monoclonal antibody inhibiting granulocyte macrophage colony-stimulating factor, was hypothesized to downregulate the granulomatous response in pulmonary sarcoidosis.METHODSRESOLVE-Lung was a double-blind, placebo-controlled trial in participants with chronic active pulmonary sarcoidosis on oral corticosteroids (OCS) and/or immunosuppressive therapies (ISTs). Participants were randomized (1:1) to receive 150 mg namilumab or placebo via subcutaneous injection every 4 weeks and were required to stop ISTs and/or taper OCS. Endpoints were assessed at week 26. Primary endpoint was proportion of participants with a rescue event, such as disease worsening necessitating treatment. Secondary endpoints included safety, lung function assessments, and corticosteroid burden.TRIAL REGISTRATIONNCT05314517.FINDINGSOf 209 participants screened, 107 were randomized to receive namilumab (n=53) or placebo (n=54). Proportion of participants with a rescue event was higher for the namilumab group (37·5%) compared with the placebo group (23·5%); stratified difference of 13·6% (90% CI: -1·5 to 28·7; p=0·12). LS mean change from baseline in ppFVC was -3·3% (90% CI: -5.1 to -1.5) for namilumab and -2·9% (90% CI: -4.5 to -1.3) for placebo (LS mean difference: -0·4%, 90% CI: -2·7 to 2·0). Proportion of participants successfully achieving OCS taper was 53·3% for namilumab and 76·9% for placebo (difference -19·4% [90% CI: -47·5 to 8·7]). Treatment-emergent adverse events were reported in 94% in both groups, most being mild or moderate in severity.INTERPRETATIONIn participants with chronic active pulmonary sarcoidosis, namilumab did not provide clinical benefit.FUNDINGKinevant Sciences GmbH.
{"title":"Safety and efficacy of namilumab for the treatment of chronic pulmonary sarcoidosis (RESOLVE-Lung): A randomized, double-blinded, multicenter, Phase 2 study.","authors":"Bernt Van Den Blink,Surinder S Birring,Nesrin Mogulkoc,Sibel N Atis,Rohit Gupta,Julien Guiot,Simon P Hart,Eva M Carmona Porquera,Hajira B Koeller,Natasha Arocho,Chantal M Petit,Jill Denning,Min Lin,Robert P Baughman,Marlies Wijsenbeek-Lourens,Theodore F Reiss, ","doi":"10.1183/13993003.01874-2025","DOIUrl":"https://doi.org/10.1183/13993003.01874-2025","url":null,"abstract":"BACKGROUNDTreatment options for chronic pulmonary sarcoidosis are limited in efficacy and by tolerability concerns. Namilumab, an investigational humanized monoclonal antibody inhibiting granulocyte macrophage colony-stimulating factor, was hypothesized to downregulate the granulomatous response in pulmonary sarcoidosis.METHODSRESOLVE-Lung was a double-blind, placebo-controlled trial in participants with chronic active pulmonary sarcoidosis on oral corticosteroids (OCS) and/or immunosuppressive therapies (ISTs). Participants were randomized (1:1) to receive 150 mg namilumab or placebo via subcutaneous injection every 4 weeks and were required to stop ISTs and/or taper OCS. Endpoints were assessed at week 26. Primary endpoint was proportion of participants with a rescue event, such as disease worsening necessitating treatment. Secondary endpoints included safety, lung function assessments, and corticosteroid burden.TRIAL REGISTRATIONNCT05314517.FINDINGSOf 209 participants screened, 107 were randomized to receive namilumab (n=53) or placebo (n=54). Proportion of participants with a rescue event was higher for the namilumab group (37·5%) compared with the placebo group (23·5%); stratified difference of 13·6% (90% CI: -1·5 to 28·7; p=0·12). LS mean change from baseline in ppFVC was -3·3% (90% CI: -5.1 to -1.5) for namilumab and -2·9% (90% CI: -4.5 to -1.3) for placebo (LS mean difference: -0·4%, 90% CI: -2·7 to 2·0). Proportion of participants successfully achieving OCS taper was 53·3% for namilumab and 76·9% for placebo (difference -19·4% [90% CI: -47·5 to 8·7]). Treatment-emergent adverse events were reported in 94% in both groups, most being mild or moderate in severity.INTERPRETATIONIn participants with chronic active pulmonary sarcoidosis, namilumab did not provide clinical benefit.FUNDINGKinevant Sciences GmbH.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"15 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147439207","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Backgrounds: Fibrosis may coexist with emphysema in chronic obstructive pulmonary disease (COPD), but computed tomography (CT) quantification is challenging. Persistent homology (PH), a topological data analysis technique, provides interpretable structural features in grayscale images. By using PH, this CT study aimed to quantify fibrotic lesions in nonemphysematous and emphysematous lungs and to investigate clinical implications of PH-based fibrosis quantification in patients with COPD.
Methods: The study included subjects from the Lung Cancer Screening (LCS) cohort (n=346) and two prospective COPD cohorts (Kyoto University [KU], n=234; Kyoto-Himeji, n=166). Based on CT value patterns and spatial topology, PH assigned each voxel as fibrotic or non-fibrotic and calculated the percentage of fibrotic lung volume (PH-fibrosis%) in association with visually identified interstitial lung abnormality (ILA) and COPD outcomes.
Results: Higher PH-fibrosis% was associated with ILAs in the LCS and KU cohorts. The two COPD cohorts consistently showed significant associations between higher baseline PH-fibrosis% and future exacerbation risk independent of emphysema and airway wall thickness (hazard ratios [HR]: 1.39 and 3.35 for KU and Kyoto-Himeji, respectively). In the KU cohort, higher PH-fibrosis% was significantly associated with increased mortality (HR: 1.94), with a similar trend in the Kyoto-Himeji cohort (HR: 1.86). Longitudinal increases in PH-fibrosis% over median 4.98 years were associated with higher exacerbation frequency and patients experiencing greater increase in PH-fibrosis% subsequently exhibited higher mortality in the KU cohort.
Conclusions: PH can be used to quantify fibrotic lesions on CT and PH-fibrosis% could be a prognostic imaging marker in patients with COPD.
{"title":"Persistent homology analysis of longitudinal CT fibrotic features in COPD.","authors":"Yusuke Shiraishi, Naoya Tanabe, Shizuo Kaji, Ryo Sakamoto, Tomoki Maetani, Yoshihiro Seri, Satoru Terada, Kunihiko Terada, Tsuyoshi Oguma, Motonari Fukui, Shigeo Muro, Tomohiro Handa, Susumu Sato, Toyohiro Hirai","doi":"10.1183/13993003.01630-2025","DOIUrl":"10.1183/13993003.01630-2025","url":null,"abstract":"<p><strong>Backgrounds: </strong>Fibrosis may coexist with emphysema in chronic obstructive pulmonary disease (COPD), but computed tomography (CT) quantification is challenging. Persistent homology (PH), a topological data analysis technique, provides interpretable structural features in grayscale images. By using PH, this CT study aimed to quantify fibrotic lesions in nonemphysematous and emphysematous lungs and to investigate clinical implications of PH-based fibrosis quantification in patients with COPD.</p><p><strong>Methods: </strong>The study included subjects from the Lung Cancer Screening (LCS) cohort (n=346) and two prospective COPD cohorts (Kyoto University [KU], n=234; Kyoto-Himeji, n=166). Based on CT value patterns and spatial topology, PH assigned each voxel as fibrotic or non-fibrotic and calculated the percentage of fibrotic lung volume (PH-fibrosis%) in association with visually identified interstitial lung abnormality (ILA) and COPD outcomes.</p><p><strong>Results: </strong>Higher PH-fibrosis% was associated with ILAs in the LCS and KU cohorts. The two COPD cohorts consistently showed significant associations between higher baseline PH-fibrosis% and future exacerbation risk independent of emphysema and airway wall thickness (hazard ratios [HR]: 1.39 and 3.35 for KU and Kyoto-Himeji, respectively). In the KU cohort, higher PH-fibrosis% was significantly associated with increased mortality (HR: 1.94), with a similar trend in the Kyoto-Himeji cohort (HR: 1.86). Longitudinal increases in PH-fibrosis% over median 4.98 years were associated with higher exacerbation frequency and patients experiencing greater increase in PH-fibrosis% subsequently exhibited higher mortality in the KU cohort.</p><p><strong>Conclusions: </strong>PH can be used to quantify fibrotic lesions on CT and PH-fibrosis% could be a prognostic imaging marker in patients with COPD.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147304036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-03-05DOI: 10.1183/13993003.02273-2025
T Douglas Bradley,Alexander G Logan,George Tomlinson,John S Floras
{"title":"Comment on ERS/ESRS statement on adaptive servo-ventilation for treatment of central sleep apnoea.","authors":"T Douglas Bradley,Alexander G Logan,George Tomlinson,John S Floras","doi":"10.1183/13993003.02273-2025","DOIUrl":"https://doi.org/10.1183/13993003.02273-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"18 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147359202","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2026-03-05DOI: 10.1183/13993003.02463-2025
Thomas Radtke,Luis Chávez,Lauren Duggan,Sanja Stanojevic,Piergiuseppe Agostoni,Paul Burns,Brenda Button,Christopher B Cooper,Jana De Brandt,Luiza Helena Degani-Costa,Monika Franczuk,Aisling McGowan,Jana Kivastik,Pierantonio Laveneziana,Zoe L Saynor,Irene Steenbruggen,Helge Hebestreit,Karl P Sylvester,
BACKGROUNDCardiopulmonary exercise testing (CPET) assesses physiological responses to incremental exercise and identifies potential causes of exercise limitation. There have been several population specific reference equations published, none that span the human age range. It would be advantageous to have all-age global reference ranges. This Task Force aimed to derive Global Lung Function Initiative (GLI) reference equations for peak oxygen uptake (VO2peak) and peak work rate (Wpeak) in healthy individuals.METHODSCPET data were collected retrospectively. Generalised additive models of location, shape and scale (GAMLSS) were used to develop reference ranges, including age, sex, height, and weight as explanatory variables. The influence of geographic region, equipment, testing protocols and averaging methods for peak exercise data was also examined.RESULTSData from 5956 healthy individuals between 6 and 83 years across 17 sites in Europe, North and South America, and Asia were analysed. There was substantial between-subject variability in both VO2peak and Wpeak, with wide confidence intervals across age groups. Heterogeneity in VO2peak was related to geographic region, metabolic cart type, and averaging methods for peak exercise values. Controlling for these variables improved model fit but not sufficiently to be reliable predictors for reference ranges.CONCLUSIONSignificant heterogeneity in CPET testing methodology and outcomes between sites precluded the development of reference ranges for VO2peak and Wpeak. This Task Force has developed a framework for prospective data collection with strictly standardised protocols and centralised data analysis to reduce variability and establish robust, clinically meaningful reference ranges for CPET outcomes.
{"title":"ERS technical standard - Reference values for cardiopulmonary exercise testing: summary report and a call for action.","authors":"Thomas Radtke,Luis Chávez,Lauren Duggan,Sanja Stanojevic,Piergiuseppe Agostoni,Paul Burns,Brenda Button,Christopher B Cooper,Jana De Brandt,Luiza Helena Degani-Costa,Monika Franczuk,Aisling McGowan,Jana Kivastik,Pierantonio Laveneziana,Zoe L Saynor,Irene Steenbruggen,Helge Hebestreit,Karl P Sylvester, ","doi":"10.1183/13993003.02463-2025","DOIUrl":"https://doi.org/10.1183/13993003.02463-2025","url":null,"abstract":"BACKGROUNDCardiopulmonary exercise testing (CPET) assesses physiological responses to incremental exercise and identifies potential causes of exercise limitation. There have been several population specific reference equations published, none that span the human age range. It would be advantageous to have all-age global reference ranges. This Task Force aimed to derive Global Lung Function Initiative (GLI) reference equations for peak oxygen uptake (VO2peak) and peak work rate (Wpeak) in healthy individuals.METHODSCPET data were collected retrospectively. Generalised additive models of location, shape and scale (GAMLSS) were used to develop reference ranges, including age, sex, height, and weight as explanatory variables. The influence of geographic region, equipment, testing protocols and averaging methods for peak exercise data was also examined.RESULTSData from 5956 healthy individuals between 6 and 83 years across 17 sites in Europe, North and South America, and Asia were analysed. There was substantial between-subject variability in both VO2peak and Wpeak, with wide confidence intervals across age groups. Heterogeneity in VO2peak was related to geographic region, metabolic cart type, and averaging methods for peak exercise values. Controlling for these variables improved model fit but not sufficiently to be reliable predictors for reference ranges.CONCLUSIONSignificant heterogeneity in CPET testing methodology and outcomes between sites precluded the development of reference ranges for VO2peak and Wpeak. This Task Force has developed a framework for prospective data collection with strictly standardised protocols and centralised data analysis to reduce variability and establish robust, clinically meaningful reference ranges for CPET outcomes.","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"67 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-03-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147359204","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
京公网安备 11010802042870号
京ICP备2023020795号-1
扫码关注我们
求助内容：
应助结果提醒方式：