Pub Date : 2026-01-22Print Date: 2026-01-01DOI: 10.1183/13993003.00665-2025
Luai Khalaili, Stefano Aliberti, Kateryna Viligorska, Francesco Blasi, Nili Stein, Raya Cohen, Rafea Zoubi, Yochai Adir, Alessandro De Angelis, Benjamin Jaaming New, Lewis Marshall, James D Chalmers, Michal Shteinberg
Background: Young adults with paediatric-onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterised. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).
Methods: Data from four EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) centres were analysed. POBE was defined as patient-reported symptom onset before the age of 18 years, while AOBE was defined as symptom onset at or after the age of 18 years. We compared POBE to AOBE and used multivariable models to identify factors associated with disease severity, including lung function, Pseudomonas aeruginosa infection, exacerbations and hospitalisations.
Results: Among 1422 patients, 249 (17.5%) had POBE (mean onset age: 6.5 years) and 1173 had AOBE (mean onset age: 55.4 years). POBE patients were younger at enrolment (50.3 versus 66 years), had longer disease duration (43.3 versus 10.8 years), worse lung function (forced expiratory volume in 1 s (FEV1): 70.8% versus 84.2% predicted), greater radiological extent (Reiff score: 6.0 versus 4.4), higher bacterial infection rates (72.3% versus 54.6%) and more exacerbations (median: 2 versus 1 per year) compared to AOBE (p<0.001 across all comparisons). Symptom duration was independently associated with P. aeruginosa infection, hospitalisation, exacerbations and reduced FEV1 % pred. Congenital aetiologies, such as primary ciliary dyskinesia and primary immunodeficiencies, further contributed to disease severity.
Conclusions: Adults with POBE exhibit greater disease severity than those with AOBE, likely due to prolonged symptom duration and congenital aetiologies. Conventional bronchiectasis severity scores may underestimate severity in young people with POBE. Optimised care, including structured transition to adult care, may mitigate progression in POBE patients.
{"title":"Greater disease severity in adults with paediatric-onset <i>versus</i> adult-onset bronchiectasis: a multicentre EMBARC registry study.","authors":"Luai Khalaili, Stefano Aliberti, Kateryna Viligorska, Francesco Blasi, Nili Stein, Raya Cohen, Rafea Zoubi, Yochai Adir, Alessandro De Angelis, Benjamin Jaaming New, Lewis Marshall, James D Chalmers, Michal Shteinberg","doi":"10.1183/13993003.00665-2025","DOIUrl":"10.1183/13993003.00665-2025","url":null,"abstract":"<p><strong>Background: </strong>Young adults with paediatric-onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterised. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).</p><p><strong>Methods: </strong>Data from four EMBARC (European Multicentre Bronchiectasis Audit and Research Collaboration) centres were analysed. POBE was defined as patient-reported symptom onset before the age of 18 years, while AOBE was defined as symptom onset at or after the age of 18 years. We compared POBE to AOBE and used multivariable models to identify factors associated with disease severity, including lung function, <i>Pseudomonas aeruginosa</i> infection, exacerbations and hospitalisations.</p><p><strong>Results: </strong>Among 1422 patients, 249 (17.5%) had POBE (mean onset age: 6.5 years) and 1173 had AOBE (mean onset age: 55.4 years). POBE patients were younger at enrolment (50.3 <i>versus</i> 66 years), had longer disease duration (43.3 <i>versus</i> 10.8 years), worse lung function (forced expiratory volume in 1 s (FEV<sub>1</sub>): 70.8% <i>versus</i> 84.2% predicted), greater radiological extent (Reiff score: 6.0 <i>versus</i> 4.4), higher bacterial infection rates (72.3% <i>versus</i> 54.6%) and more exacerbations (median: 2 <i>versus</i> 1 per year) compared to AOBE (p<0.001 across all comparisons). Symptom duration was independently associated with <i>P. aeruginosa</i> infection, hospitalisation, exacerbations and reduced FEV<sub>1</sub> % pred. Congenital aetiologies, such as primary ciliary dyskinesia and primary immunodeficiencies, further contributed to disease severity.</p><p><strong>Conclusions: </strong>Adults with POBE exhibit greater disease severity than those with AOBE, likely due to prolonged symptom duration and congenital aetiologies. Conventional bronchiectasis severity scores may underestimate severity in young people with POBE. Optimised care, including structured transition to adult care, may mitigate progression in POBE patients.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":21.0,"publicationDate":"2026-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12824631/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144559608","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}
Pub Date : 2026-01-22DOI: 10.1183/13993003.01886-2025
Glauco M Plens,Ewan C Goligher
{"title":"Oxygenation and ventilatory variables in ARDS: time to revise our definitions of severity?","authors":"Glauco M Plens,Ewan C Goligher","doi":"10.1183/13993003.01886-2025","DOIUrl":"https://doi.org/10.1183/13993003.01886-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"69 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146021440","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 : 2026-01-15DOI: 10.1183/13993003.02207-2025
Andrea Baccelli,Gulammehdi Haji,Hannah Tighe,Luke S Howard
{"title":"Safety of Cardiopulmonary Exercise Testing in Pulmonary Hypertension: Insights from a UK Tertiary Referral Centre.","authors":"Andrea Baccelli,Gulammehdi Haji,Hannah Tighe,Luke S Howard","doi":"10.1183/13993003.02207-2025","DOIUrl":"https://doi.org/10.1183/13993003.02207-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"5 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145986620","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 : 2026-01-15DOI: 10.1183/13993003.01988-2025
Kevin I Duan,Chris Carlsten,Kate M Johnson,Michael R Law
{"title":"Early Impacts of Individual Cigarette Health Warnings in Canada.","authors":"Kevin I Duan,Chris Carlsten,Kate M Johnson,Michael R Law","doi":"10.1183/13993003.01988-2025","DOIUrl":"https://doi.org/10.1183/13993003.01988-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"37 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145986616","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 : 2026-01-15DOI: 10.1183/13993003.02312-2025
Christopher J Ryerson,Sara Piciucchi,William D Travis,Andrew G Nicholson,Ayodeji Adegunsoye
{"title":"Clinical Applications of the 2025 ERS/ATS Statement Update of the International Multidisciplinary Classification of the Interstitial Pneumonias.","authors":"Christopher J Ryerson,Sara Piciucchi,William D Travis,Andrew G Nicholson,Ayodeji Adegunsoye","doi":"10.1183/13993003.02312-2025","DOIUrl":"https://doi.org/10.1183/13993003.02312-2025","url":null,"abstract":"","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":"58 1","pages":""},"PeriodicalIF":24.3,"publicationDate":"2026-01-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145986619","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}
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