Aetiologies and outcomes of pediatric-onset bronchiectasis in adults: data from the EMBARC registry

<bold>Background:</bold> Pediatric and adult bronchiectasis have different clinical profiles but data on long-term follow-up of children with bronchiectasis are scarce. We compared the aetiologies and outcomes of adults with pediatric-onset (paed-BE) bronchiectasis compared with adult-onset (adult-BE) bronchiectasis in the EMBARC registry. <bold>Methods:</bold> Patients ≤47 years old were included. People with paed-BE bronchiectasis were defined as adults with a bronchiectasis diagnosis made ≥20 years ago, and adult-BE as diagnosis made <20 years ago. We compared data on demography, aetiology and severity measures between paed-BE and adult-BE. <bold>Results:</bold> 663 adults with paed-BE and 1237 with adult- BE were included. Compared to adult-BE, Paed-BE were younger (median (IQR) 28(22-36) vs. 37(32-41) years) and more likely to be never smokers (85.7% vs. 66.6%, p<.001). Rhinosinusitis and nasal polyps were more common in paed-BE than in adult-BE (41.6% vs. 27.6% and 18.8% vs. 10.3%, respectively, p<.001) while asthma was more common in adult- BE (36.4% vs. 26.8%). Paed-BE were more likely to have an aetiology of PCD: 29.4% vs. 6.8% (p<.001), and less like to have an idiopathic aetiology (27.5% vs. 37.2%, p<.001). Patients with paed-BE had lower lung function and worse severity than adult- BE: median FEV<sub>1</sub>% 74.0(50.1-91.3) vs. 84.6(63.4-100.7), BSI 4(2-8) vs. 3(1-6). Paed-BE had a higher risk of exacerbations when adjusted for age, sex, <italic>Pseudomonas</italic> infection, %FEV<sub>1</sub>, Inhaled antibiotic use, macrolide use, asthma and smoking: HR 1.59(1.30-1.94). <bold>Conclusions:</bold> despite younger age, patients with paed-BE have worse outcomes than adult- BE. This may be attributed to different aetiologies or to longer disease duration.

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.pa2391
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1
Primary Topic
Cystic Fibrosis Research Advances
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article
Field-Weighted Citation Impact
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article

Aetiologies and outcomes of pediatric-onset bronchiectasis in adults: data from the EMBARC registry

Francesco Blasi, Josje Altenburg, Michal Shteinberg, Pieter Goemmine et al.
1 citations
Cystic Fibrosis Research Advances
0.42
article

Aetiologies and outcomes of pediatric-onset bronchiectasis in adults: data from the EMBARC registry

Francesco Blasi, Josje Altenburg, Michal Shteinberg, Pieter Goemmine, Charles Haworth, J.S. Elborn, Felix C. Ringshausen, Eva Polverino, Katerina Dimakou, Rosario Menéndez, James Duncan Chalmers, Montserrat Vendrell, Anthony De Soyza, Pierre‐Régis Burgel, Stefano Aliberti, Antoní Torres, Megan Crichton, Marlène Murris, Adam Hill, Michael Loebingher, Wim Borsma
article en
1 citations

Abstract

<bold>Background:</bold> Pediatric and adult bronchiectasis have different clinical profiles but data on long-term follow-up of children with bronchiectasis are scarce. We compared the aetiologies and outcomes of adults with pediatric-onset (paed-BE) bronchiectasis compared with adult-onset (adult-BE) bronchiectasis in the EMBARC registry. <bold>Methods:</bold> Patients ≤47 years old were included. People with paed-BE bronchiectasis were defined as adults with a bronchiectasis diagnosis made ≥20 years ago, and adult-BE as diagnosis made <20 years ago. We compared data on demography, aetiology and severity measures between paed-BE and adult-BE. <bold>Results:</bold> 663 adults with paed-BE and 1237 with adult- BE were included. Compared to adult-BE, Paed-BE were younger (median (IQR) 28(22-36) vs. 37(32-41) years) and more likely to be never smokers (85.7% vs. 66.6%, p<.001). Rhinosinusitis and nasal polyps were more common in paed-BE than in adult-BE (41.6% vs. 27.6% and 18.8% vs. 10.3%, respectively, p<.001) while asthma was more common in adult- BE (36.4% vs. 26.8%). Paed-BE were more likely to have an aetiology of PCD: 29.4% vs. 6.8% (p<.001), and less like to have an idiopathic aetiology (27.5% vs. 37.2%, p<.001). Patients with paed-BE had lower lung function and worse severity than adult- BE: median FEV<sub>1</sub>% 74.0(50.1-91.3) vs. 84.6(63.4-100.7), BSI 4(2-8) vs. 3(1-6). Paed-BE had a higher risk of exacerbations when adjusted for age, sex, <italic>Pseudomonas</italic> infection, %FEV<sub>1</sub>, Inhaled antibiotic use, macrolide use, asthma and smoking: HR 1.59(1.30-1.94). <bold>Conclusions:</bold> despite younger age, patients with paed-BE have worse outcomes than adult- BE. This may be attributed to different aetiologies or to longer disease duration.

Queen's University Belfast (GB), Humanitas University (IT), Inserm (FR), Université Paris Cité (FR), Royal Papworth Hospital (GB), Ninewells Hospital (GB), Harefield Hospital (GB), Edinburgh Royal Infirmary (GB), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (IT), Centre Hospitalier Universitaire de Toulouse (FR), Medizinische Hochschule Hannover (DE), Assistance Publique – Hôpitaux de Paris (FR), Northwest Hospital (US), Institut d'Investigació Biomèdica de Girona (ES), Hospital Clínic de Barcelona (ES), Rappaport Family Institute for Research in the Medical Sciences (IL), Vall d'Hebron Hospital Universitari (ES), NIHR Newcastle Biomedical Research Centre (GB), Institut Cochin (FR), Hospital Universitari de Girona Doctor Josep Trueta (ES), IRCCS Humanitas Research Hospital (IT), AZ Sint-Lucas (BE), Instituto de Investigación Sanitaria La Fe (ES), Sotiria General Hospital (GR), Universitat de Barcelona (ES), University of Amsterdam (NL)
Openalex Percentile: Top 31%
Cystic Fibrosis Research Advances
0.42
DOISourceOpenAlex
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