Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC).

BACKGROUND

Bronchiectasis is a heterogeneous, neglected disease with few multicentre studies exploring the causes, severity, microbiology, and treatment of the disease across Europe. This aim of this study was to describe the clinical characteristics of bronchiectasis and compare between different European countries.

METHODS

EMBARC is an international clinical research network for bronchiectasis. We report on a multicentre, prospective, observational, non-interventional, cohort study (the EMBARC registry) conducted across 27 European countries and Israel. Comprehensive clinical data were collected from adult patients (aged ≥18 years) at baseline and annual follow-up visits using electronic case report form. Data from individual countries were grouped into four regions (the UK, northern and western Europe, southern Europe, and central and eastern Europe according to modified EU EuroVoc classification). Follow-up data were used to explore differences in exacerbation frequency between regions using a negative binomial regression model.

FINDINGS

Between Jan 12, 2015, and April 12, 2022, 16 963 individuals were enrolled. Median age was 67 years (IQR 57-74), 10 335 (60·9%) participants were female and 6628 (39·1%) were male. The most common cause of bronchiectasis in all 16 963 participants was post-infective disease in 3600 (21·2%); 6466 individuals (38·1%) were classified as idiopathic. Individuals with bronchiectasis experienced a median of two exacerbations (IQR 1-4) per year and 4483 (26·4%) patients had a hospitalisation for exacerbation in the previous year. When examining the percentage of all isolated bacteria, marked differences in microbiology were seen between countries, with a higher frequency of Pseudomonas aeruginosa and lower Haemophilus influenzae frequency in southern Europe, compared with higher H influenzae in the UK and northern and western Europe. Compared with other regions, patients in central and eastern Europe had more severe bronchiectasis measured by the Bronchiectasis Severity Index (51·3% vs 35·1% in the overall cohort) and more exacerbations leading to hospitalisations (57·9% vs 26·4% in the overall cohort). Overall, patients in central and eastern Europe had an increased frequency of exacerbations (adjusted rate ratio [RR] 1·12, 95% CI 1·01-1·25) and a higher frequency of exacerbations leading to hospitalisations (adjusted RR 1·71, 1·44-2·02) compared with patients in other regions. Treatment of bronchiectasis was highly heterogeneous between regions.

INTERPRETATION

Bronchiectasis shows important geographical variation in causes, microbiology, severity, and outcomes across Europe.

FUNDING

European Union-European Federation of Pharmaceutical Industries and Associations Innovative Medicines Initiative.

TRANSLATIONS

For the Arabic, French, German, Greek, Hebrew, Irish, Russian and Spanish translations of the abstract see Supplementary Materials section.

Informations de copyright

Copyright © 2023 The Author(s). Published by Elsevier Ltd. This is an Open Access article under the CC BY 4.0 license. Published by Elsevier Ltd.. All rights reserved.

Déclaration de conflit d'intérêts

Declaration of interests JC reports grants or contracts from Grifols; consulting fees from Antabio, AstraZeneca, Boehringer Ingelheim, Chiesi, Glaxosmithkline, Grifols, Insmed, Janssen, Novartis, Pfizer, and Zambon; and leadership or fiduciary roles as Chair of European Respiratory Society (ERS) Bronchiectasis Guideline Task Force, Chief Editor of European Respiratory Journal, and Chair of EMBARC Clinical Research Collaboration. EP reports grants or contracts from Grifols; consulting fees from Bayer, Zambon, Pfizer, Chiesi, Shire, Shionogi, Insmed, Moderna, and Pfizer; payment for expert testimony from Chiesi; leadership or fiduciary roles as ERS Task Force for Bronchiectasis Guidelines, co-chair of EMBARC; and receipt of equipment, materials, drugs, medical writing, gifts, or other services from Zambon. FR reports grants or contracts from German Center for Lung Research (DZL), German Center for Infection Research (EU and European Federation of Pharmaceutical Industries and Associations [EFPIA]) and iABC Consortium (including Alaxia, Basilea, Novartis, and Polyphor), Mukoviszidose Institute, Novartis, Insmed Germany, Grifols, Bayer, and InfectoPharm, to the institution; consulting fees from Parion, Grifols, Zambon, Insmed, Helmholtz-Zentrum für Infektionsforschung; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from I!DE Werbeagentur, Interkongress, AstraZeneca, Insmed, Grifols, and Universitätsklinikum Frankfurt am Main; payment for expert testimony from Social Court Cologne, to the institution; support for attending meetings or travel from German Kartagener Syndrome and PCD PAG, and Mukoviszidose; participation on a Data Safety Monitoring Board or Advisory Board for Insmed, Grifols, and Shionogi; leadership or fiduciary role in other board, society, committee, or advocacy groups as Coordinator of the ERN-LUNG Bronchiectasis Core Network; Chair of the German Bronchiectasis Registry PROGNOSIS; Member of the SteerCo of the European Bronchiectasis Registry EMBARC; Member of the SteerCo of the European Non-tuberculous Mycobacterial registry EMBARC-NTM; Co-Speaker of the Medical Advisory Board of the German Kartagener Syndrome and PCD PAG; Speaker of the Respiratory Infections and tuberculosis group of the German Respiratory Society (DGP; Speaker of the Cystic Fibrosis group of DGP; Prinicipal Investigator for DZL; Member of the Protocol Review Committee of the PCD-CTN; Member of Physician Association of the German Cystic Fibrosis PAG; and fees for clinical trial participation paid to the institution from AstraZeneca, Boehringer Ingelheim, Celtaxsys, Corbus, Insmed, Novartis, Parion, University of Dundee, Vertex, and Zambon. ADS reports grants or contracts from Bayer, Gilead, Pfizer, Astrazeneca, and Novartis, outside of the submitted work; consulting fees from Boehringer, Bayer, Gilead, Pfizer, GSK, and Astrazeneca, outside of the submitted work; support for attending meetings or travel from AstraZeneca, Chiesi, and GSK; and participation on a Data Safety Monitoring Board or Advisory Board for Bayer. MV reports participation in EMBARC registry; grants or contracts from Chiesi; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Insmed and Publi creation; support for attending meetings or travel from Zambon, Chiesi, Novartis, Behring; participation on a Data Safety Monitoring Board or Advisory Board for Insmed; and receipt of equipment, materials, drugs, medical writing, gifts, or other services from Insmed and Novartis. PRB reports grants or contracts from GSK and Vertex, to the institution; consulting fees from Insmed, Vertex, Viatris, and Zambon; and participation on a Data Safety Monitoring Board or Advisory Board for Insmed. CH reports grants or contracts from the National Institute for Health and Care Research (NIHR 2020, NIHR133876 Seven versus Fourteen Days Antibiotics for Patients with Bronchiectasis Requiring Intravenous Antibiotics to the institution and NIHR [2020–2023] Bronchodilators and corticosteroids in bronchiectasis) to the institution; consulting fees from 30 Technology, CSL Behring, Chiesi, Insmed, Janssen, LifeArc, Meiji, Mylan, Novartis, Pneumagen, Shionogi, and Zambon; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Zambon, Insmed, Chiesi, and 30 Technology. ML reports consulting fees from Armata, 30T, AstraZeneca, Parion, Insmed, Chiesi, Zambon, Electromed, Recode, AN2, and Savara; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from Insmed; and the ERS Infection Group chair role. KD reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Pfizer, Novartis, AstraZeneca, Boehringer Ingelheim, Chiesi, and Norma Hellas; and upport for attending meetings or travel from Novartis, AstraZeneca, Boehringer Ingelheim, Chiesi, Pfizer, Norma Hellas, and Menarini. MM reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Pfizer; payment for expert testimony from Vertex; support for attending meetings or travel from Zambon; and participation on a Data Safety Monitoring Board or Advisory Board from Zambon, and Viatris. TW reports grants or contracts from German Ministry of Research and Education and consulting fees from AstraZeneca, GSK, and Insmed. FB reports grants or contracts from AstraZeneca, Chiesi, GSK, Insmed; consulting fees from GSK, Menarini, OM Pharma; and payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from AstraZeneca, Chiesi, GSK, Grifols, Insmed, Menarini, Novartis, Ompharma, Pfizer, Sanofi, Vertex, Viatris, and Zambon. MS reports grants or contracts from GSK and Trudell pharma; consulting fees from AstraZeneca, Boehringer Ingelheim, Dexcel, Kamada, Synchrony Medical, Trumed, and Zambon; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing, or educational events from AstraZeneca, Boehringer Ingelheim, and Kamada; support for attending meetings or travel from Boehringer Ingelheim, AstraZeneca, and Kamada; participation on a Data Safety Monitoring Board or Advisory Board for Bonus Biotherapeutics, Boehringer Ingelheim, and AstraZeneca; leadership or fiduciary role in other board, society, committee, or advocacy group with Israeli Pulmonology Society, Israeli Society for Tuberculosis and Mycobacterial Diseases, and EMBARC as unpaid management board member and ERJ, Chest as unpaid Editorial board member; and receipt of equipment, materials, drugs, medical writing, gifts, or other services from Trudell Medical International. JSE reports grants or contracts from EU FP7 with Novartis and Polyphor; and consulting fees from Vertex. PG reports payment or honoraria for a lecture on bronchiectasis from GSK. SA reports grants or contracts from Insmed, Chiesi, Fisher & Paykel, to the institution; royalties or licenses with McGraw Hill; consulting fees from Insmed Incorporated, Insmed Italy, Insmed Ireland, Zambon, AstraZeneca UK, AstraZeneca Pharmaceutical, CSL Behring, Grifols, Fondazione Charta, Boehringer Ingelheim, Chiesi Farmaceutici Spa, Zcube, MENARINI, MSD Italia, Physioassist SAS, and GlaxoSmithKline Spa; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from GlaxoSmithKline Spa, Thermofisher Scientific, Insmed Italy, Insmed Ireland, Zambon, and Fondazione Internazionale Menarini; and participation on a Data Safety Monitoring Board or Advisory Board for Insmed Incorporated, Insmed Italy, AstraZeneca UK, and MSD Italia. All other authors declare no competing interests.