Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis: a multicenter EMBARC registry study

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; M. Shteinberg

doi:10.1183/13993003.00665-2025

Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis: a multicenter EMBARC registry study

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, M. Shteinberg (Lead / Corresponding author)

Respiratory Medicine and Gastroenterology

Research output: Contribution to journal › Article › peer-review

Abstract

BACKGROUND: Young adults with paediatric- onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterized. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).

METHODS: Data from four EMBARC (European Bronchiectasis Registry) centers were analyzed. POBE was defined as patient-reported symptom onset before 18 years, while AOBE was defined as symptom onset at or after 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 hospitalizations.

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 enrollment (50.3 versus 66 years), had longer disease duration (43.3 versus 10.8 years), worse lung function (FEV1% predicted: 70.8 versus 84.2), greater radiological extent (Reiff index: 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, hospitalization, exacerbations, and reduced FEV1%. Congenital etiologies, such as primary ciliary dyskinesia and primary immune deficiencies, 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. Optimized care, including structured transition to adult care, may mitigate progression in POBE patients.

Original language	English
Journal	The European respiratory journal
Early online date	3 Jul 2025
DOIs	https://doi.org/10.1183/13993003.00665-2025
Publication status	E-pub ahead of print - 3 Jul 2025

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Khalaili, L., Aliberti, S., Viligorska, K., Blasi, F., Stein, N., Cohen, R., Zoubi, R., Adir, Y., De Angelis, A., Jaaming New, B., Marshall, L., Chalmers, J. D., & Shteinberg, M. (2025). Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis: a multicenter EMBARC registry study. The European respiratory journal. Advance online publication. https://doi.org/10.1183/13993003.00665-2025

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title = "Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis: a multicenter EMBARC registry study",

abstract = "BACKGROUND: Young adults with paediatric- onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterized. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).METHODS: Data from four EMBARC (European Bronchiectasis Registry) centers were analyzed. POBE was defined as patient-reported symptom onset before 18 years, while AOBE was defined as symptom onset at or after 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 hospitalizations.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 enrollment (50.3 versus 66 years), had longer disease duration (43.3 versus 10.8 years), worse lung function (FEV1\% predicted: 70.8 versus 84.2), greater radiological extent (Reiff index: 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, hospitalization, exacerbations, and reduced FEV1\%. Congenital etiologies, such as primary ciliary dyskinesia and primary immune deficiencies, 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. Optimized care, including structured transition to adult care, may mitigate progression in POBE patients.",

author = "Luai Khalaili and Stefano Aliberti and Kateryna Viligorska and Francesco Blasi and Nili Stein and Raya Cohen and Rafea Zoubi and Yochai Adir and \{De Angelis\}, Alessandro and \{Jaaming New\}, Benjamin and Lewis Marshall and Chalmers, \{James D\} and M. Shteinberg",

note = "Copyright {\textcopyright}The authors 2025.",

year = "2025",

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doi = "10.1183/13993003.00665-2025",

language = "English",

journal = "The European respiratory journal",

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Khalaili, L, Aliberti, S, Viligorska, K, Blasi, F, Stein, N, Cohen, R, Zoubi, R, Adir, Y, De Angelis, A, Jaaming New, B, Marshall, L, Chalmers, JD & Shteinberg, M 2025, 'Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis: a multicenter EMBARC registry study', The European respiratory journal. https://doi.org/10.1183/13993003.00665-2025

TY - JOUR

T1 - Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis

T2 - a multicenter EMBARC registry study

AU - Khalaili, Luai

AU - Aliberti, Stefano

AU - Viligorska, Kateryna

AU - Blasi, Francesco

AU - Stein, Nili

AU - Cohen, Raya

AU - Zoubi, Rafea

AU - Adir, Yochai

AU - De Angelis, Alessandro

AU - Jaaming New, Benjamin

AU - Marshall, Lewis

AU - Chalmers, James D

AU - Shteinberg, M.

PY - 2025/7/3

Y1 - 2025/7/3

N2 - BACKGROUND: Young adults with paediatric- onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterized. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).METHODS: Data from four EMBARC (European Bronchiectasis Registry) centers were analyzed. POBE was defined as patient-reported symptom onset before 18 years, while AOBE was defined as symptom onset at or after 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 hospitalizations.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 enrollment (50.3 versus 66 years), had longer disease duration (43.3 versus 10.8 years), worse lung function (FEV1% predicted: 70.8 versus 84.2), greater radiological extent (Reiff index: 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, hospitalization, exacerbations, and reduced FEV1%. Congenital etiologies, such as primary ciliary dyskinesia and primary immune deficiencies, 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. Optimized care, including structured transition to adult care, may mitigate progression in POBE patients.

AB - BACKGROUND: Young adults with paediatric- onset bronchiectasis (POBE) represent a minority of bronchiectasis patients and are poorly characterized. We aimed to compare the characteristics and severity of adults with POBE to adult-onset bronchiectasis (AOBE).METHODS: Data from four EMBARC (European Bronchiectasis Registry) centers were analyzed. POBE was defined as patient-reported symptom onset before 18 years, while AOBE was defined as symptom onset at or after 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 hospitalizations.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 enrollment (50.3 versus 66 years), had longer disease duration (43.3 versus 10.8 years), worse lung function (FEV1% predicted: 70.8 versus 84.2), greater radiological extent (Reiff index: 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, hospitalization, exacerbations, and reduced FEV1%. Congenital etiologies, such as primary ciliary dyskinesia and primary immune deficiencies, 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. Optimized care, including structured transition to adult care, may mitigate progression in POBE patients.

U2 - 10.1183/13993003.00665-2025

DO - 10.1183/13993003.00665-2025

M3 - Article

C2 - 40610053

SN - 0903-1936

JO - The European respiratory journal

JF - The European respiratory journal

ER -

Greater disease severity in adults with paediatric-onset versus adult-onset bronchiectasis: a multicenter EMBARC registry study

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