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Rationale: COPD and bronchiectasis are commonly reported together. Studies report varying impacts of co-diagnosis on outcomes, which may be related to different definitions of disease used across studies. Objectives: To investigate the prevalence of chronic obstructive pulmonary disease (COPD) associated with bronchiectasis and its relationship with clinical outcomes. We further investigated the impact of implementing the standardized ROSE criteria (radiological bronchiectasis [R], obstruction [FEV 1 /FVC ratio <0.7; O], symptoms [S], and exposure [⩾10 pack-years of smoking; E]), an objective definition of the association of bronchiectasis with COPD. Methods: Analysis of the EMBARC (European Bronchiectasis Registry), a prospective observational study of patients with computed tomography-confirmed bronchiectasis from 28 countries. The ROSE criteria were used to objectively define the association of bronchiectasis with COPD. Key outcomes during a maximum of 5 years of follow-up were exacerbations, hospitalization, and mortality. Measurements and Main Results: A total of 16,730 patients with bronchiectasis were included; 4,336 had a clinician-assigned codiagnosis of COPD, and these patients had more exacerbations, worse quality of life, and higher severity scores. We observed marked overdiagnosis of COPD: 22.2% of patients with a diagnosis of COPD did not have airflow obstruction and 31.9% did not have a history of ⩾10 pack-years of smoking. Therefore, 2,157 patients (55.4%) met the ROSE criteria for COPD. Compared with patients without COPD, patients who met the ROSE criteria had increased risks of exacerbations and exacerbations resulting in hospitalization during follow-up (incidence rate ratio, 1.25; 95% confidence interval, 1.15-1.35; vs. incidence rate ratio, 1.69; 95% confidence interval, 1.51-1.90, respectively). Conclusions: The label of COPD is often applied to patients with bronchiectasis who do not have objective evidence of airflow obstruction or a smoking history. Patients with a clinical label of COPD have worse clinical outcomes.

Background: International guidelines recommend airway clearance management as one of the important pillars of bronchiectasis treatment. However, the extent to which airway clearance is used for people with bronchiectasis in Europe is unclear. The aim of the study was to identify the use of airway clearance management in patients with bronchiectasis across different countries and factors influencing airway clearance use., Methods: This was a prospective observational study using data from the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) Registry between January 2015 and April 2022. Prespecified options for airway clearance management were recorded, including airway clearance techniques, devices and use of mucoactive drugs., Results: 16 723 people with bronchiectasis from 28 countries were included in the study. The mean age was 67 years (interquartile range 57-74 years, range 18-100 years) and 61% were female. 72% of the participants reported daily sputum expectoration and 52% (95% CI 51-53%) of all participants reported using regular airway clearance management. Active cycle of breathing technique was used by 28% of the participants and airway clearance devices by 16% of participants. The frequency of airway clearance management and techniques used varied significantly between different countries. Participants who used airway clearance management had greater disease severity and worse symptoms, including a higher daily sputum volume, compared to those who did not use it regularly. Mucoactive drugs were also more likely to be used in participants with more severe disease. Access to specialist respiratory physiotherapy was low throughout Europe, but particularly low in Eastern Europe., Conclusions: Only a half of people with bronchiectasis in Europe use airway clearance management. Use of and access to devices, mucoactive drugs and specialist chest physiotherapy appears to be limited in many European countries., Competing Interests: Conflict of interest: B. Hererro-Cortina reports payment or honoraria for lectures, presentations, manuscript writing or educational events from SEPAR (Spanish Respiratory Society), and has a leadership or fiduciary role in SEPAR. S. Aliberti reports grants from Insmed Incorporated, Chiesi, Fisher and Paykel, and GSK; royalties or licences from McGraw Hill; consulting fees from Insmed Incorporated, Insmed Italy, Insmed Ireland Ltd, Zambon Spa, AstraZeneca UK Ltd, AstraZeneca Pharmaceutical LP, CSL Behring GmbH, Grifols, Fondazione Internazionale Menarini, Moderna, Chiesi, MCD Italis Srl, Brahms, Physioassist SAS and GSK SpA; payment or honoraria for lectures, presentations, manuscript writing or educational events from GSK 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 Ltd and MSD Italia Srl. P.C. Goeminne reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed, GSK and Chiesi; support for attending meetings and/or travel from Chiesi; and participation on a data safety monitoring board or advisory board for Boehringer, GSK and Pfizer. E. Polverino reports grants from Grifols; consulting fees from Insmed, Bayer, Chiesi and Zambon; payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Chiesi, Grifols, GSK, Insmed, Menarini and Zambon; and support for attending meetings and/or travel from Insmed, Pfizer and Moderna. K. Dimakou reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Novartis, Boehringer Ingelheim, GSK, Norma Hellas, Chiesi, AstraZeneca and Zambon; support for attending meetings and/or travel from Novartis, Boehringer Ingelheim, GSK, Norma Hellas, Chiesi, AstraZeneca and Menarini, and participation on a data safety monitoring board or advisory board for Novartis, GSK and Chiesi. C.S. Haworth reports payment or honoraria for lectures, presentations, manuscript writing or educational events from 30 Technology, CSL Behring, Chisi, Insmed, Janssen, LifeArc, Meiji, Mylan, Pneumagen, Shionogi, Vertex and Zambon. M.R. Loebinger reports consulting fees from Armata, 30T, AstraZeneca, Parion, Insmed, Chiesi, Zambon, Electromed, Recode, AN2 and Boehringer Ingelheim; payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed; and is ERS Infection Group Chair. A. De Soyza reports grants from AstraZeneca, Pfizer, GSK and Novartis; consulting fees from AstraZeneca, Insmed, GSK, Boehringer, 30T and Bayer; and payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, Pfizer, GSK and Novartis. M. Vendrell reports grants from Chiesi; payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed and Publi Creation; support for attending meetings and/or travel from Zambon, Chiesi, Novartis, Behring and Gebro; participation on a data safety monitoring board or advisory board for Insmed; and receipt of equipment, materials, drugs, medical writing, gifts or services from Insmed and Novartis. P.R. Burgel reports grants from GSK and Vertex; consulting fees from AstraZeneca, Chiesi, GSK, Insmed, MSD, Vertex, Viatris and Zambon; and support for attending meetings and/or travel from AstraZeneca and Chiesi. S. Sutharsan reports grants from Vertex, Galapagos, Insmed, Proteostasis and Corbus; consulting fees from Insmed, Vertex Pharmaceuticals and Boehringer Ingelheim; and payment or honoraria for lectures, presentations, manuscript writing or educational events from Vertex Pharmaceuticals, Boehringer Ingelheim and Insmed. S. Škrgat reports honoraria for educational events, invited lectures and presentations supported by Sanofi, AstraZeneca, Medis, Berlin-Chemie and Chiesi; and participation on a data safety monitoring board or advisory board for AstraZeneca. D. Stolz reports payment or honoraria for lectures, presentations, manuscript writing or educational events from CSL Behring, Berlin-Chemie, Menarini, Novartis, GSK, AstraZeneca, Vifor, Merck, Chiesi and Sanofi; and participation on a data safety monitoring board or advisory board for GSK and CSL Behring. P. Kauppi reports support for attending meetings and/or travel from Nordic Respiratory Academy; participation on a data safety monitoring board or advisory board for Swedish Orphan Biovitrium; a leadership or fiduciary role for the Finnish Respiratory Society and Finnish Tuberculosis Foundation Grant Committee; and receipt of equipment, materials, drugs, medical writing, gifts or services from Theravance. A. Bossios reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Chiesi; and is Secretary of Assembly 5 (Airway diseases, asthma, COPD and chronic cough) of the ERS, Vice-chair of the Nordic Severe Asthma Network (NSAN)-NORDSTAR and a member of the Steering Committee of the ERS CRC for severe asthma (SHARP). I. Clifton reports payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca and GSK; and participation on a data safety monitoring board or advisory board for AstraZeneca, GSK and Infex Therapeutics. M.L. Crichton reports consulting fees from Boxer Capital LLC. P. Walker is Chair of the British Thoracic Society. D. Obradovic is President of the Serbian Society of Intensive Care Medicine. A. Amorim reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Zambon Group, and support for attending meetings and or/travel from Zambon Group, Boehringer Ingelheim and Novartis Farma. F. Blasi reports grants from AstraZeneca, Chiesi and Insmed; consulting fees from Menarini; and payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, Chiesi, GSK, Guidotti, Grifols, Insmed, Menarini, Novartis, OM Pharma, Pfizer, Sanofi, Viatris, Vertex and Zambon. M. Jankovic Makek reports consulting fees from Insmed, Biomerieux and MSD; payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed, MSD, Pfizer and Teva; and support for attending meetings and/or travel from MSD, Pfizer and Berlin-Chemie. M. Shteinberg reports consulting fees from GSK, Boehringer Ingelheim, Kamada and Zambon; payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed, Boehringer Ingelheim, GSK, AstraZeneca, Teva, Novartis, Kamada and Sanofi; support for attending meetings and/or travel from Novartis, Actelion, Boehringer Ingelheim, GSK and Rafa; participation on a data safety monitoring board or advisory board for Bonus Therapeutics, Israel; leadership or fiduciary roles for EMBARC Management, Israel Pulmonology Society Board and the Israel Society for TB and Mycobacterial Diseases; receipt of equipment, materials, drugs, medical writing, gifts or services from Trudell Medical Int.; and is and Associate Editor of the American Journal of Respiratory and Critical Care Medicine. J.D. Chalmers reports grants from AstraZeneca, Boehringer Ingelheim, Genentech, Gilead Sciences, GSK, Grifols, Insmed, LifeArc and Novartis; and consulting fees from AstraZeneca, Chiesi, GSK, Insmed, Grifols, Novartis, Boehringer Ingelheim, Pfizer, Janssen, Antabio and Zambon. F.C. Ringshausen reports grants from German Center for Lung Research (DZL), German Center for Infection Research (DZIF), IMI (EU/EFPIA) and iABC Consortium (including Alaxia, Basilea, Novartis and Polyphor), Mukoviszidose Institute, Novartis, Insmed Germany, Grifols, Bayer and InfectoPharm; consulting fees from Parion, Grifols, Zambon, Insmed and Helmholtz-Zentrum für Infektionsforschung; payment or honoraria for lectures, presentations, manuscript writing or educational events from I!DE Werbeagentur GmbH, Interkongress GmbH, AstraZeneca, Insmed, Grifols and Universitätsklinikum Frankfurt am Main; payment for expert testimony from Social Court Cologne; support for attending meetings and/or travel from German Kartagener Syndrome and Primary Ciliary Dyskinesia Patient Advocacy Group Mukoviszidose e.V.; participation on a data safety monitoring board or advisory board for Insmed, Grifols and Shionogi; leadership or fiduciary roles as Coordinator of the ERN-LUNG Bronchiectasis Core Network, Chair of the German Bronchiectasis Registry PROGNOSIS, Member of the Steering Committee of the European Bronchiectasis Registry EMBARC, Member of the Steering Committee of the European Nontuberculous Mycobacterial Pulmonary Disease Registry EMBARC-NTM, Co-Speaker of the Medical Advisory Board of the German Kartagener Syndrome and PCD Patient Advocacy Group, Speaker of the Respiratory Infections and TB group of the German Respiratory Society, Speaker of the Cystic Fibrosis group of German Respiratory Society (DGP), principal investigator of the German Center for Lung Research, Member of the Protocol Review Committee of the PCD-CTN and Member of Physician Association of the German Cystic Fibrosis Patient Advocacy Group; and other financial or non-financial interests in AstraZeneca, Boehringer Ingelheim, Celtaxsys, Corbus, Insmed, Novartis, Parion, University of Dundee, Vertex and Zambon. The remaining authors have no potential conflicts of interest to disclose., (Copyright ©The authors 2024.)

Background: Asthma is commonly reported in patients with a diagnosis of bronchiectasis., Objective: The aim of this study was to evaluate whether patients with bronchiectasis and asthma (BE+A) had a different clinical phenotype and different outcomes compared with patients with bronchiectasis without concomitant asthma., Methods: A prospective observational pan-European registry (European Multicentre Bronchiectasis Audit and Research Collaboration) enrolled patients across 28 countries. Adult patients with computed tomography-confirmed bronchiectasis were reviewed at baseline and annual follow-up visits using an electronic case report form. Asthma was diagnosed by the local investigator. Follow-up data were used to explore differences in exacerbation frequency between groups using a negative binomial regression model. Survival analysis used Cox proportional hazards regression., Results: Of 16,963 patients with bronchiectasis included for analysis, 5,267 (31.0%) had investigator-reported asthma. Patients with BE+A were younger, were more likely to be female and never smokers, and had a higher body mass index than patients with bronchiectasis without asthma. BE+A was associated with a higher prevalence of rhinosinusitis and nasal polyps as well as eosinophilia and Aspergillus sensitization. BE+A had similar microbiology but significantly lower severity of disease using the bronchiectasis severity index. Patients with BE+A were at increased risk of exacerbation after adjustment for disease severity and multiple confounders. Inhaled corticosteroid (ICS) use was associated with reduced mortality in patients with BE+A (adjusted hazard ratio 0.78, 95% CI 0.63-0.95) and reduced risk of hospitalization (rate ratio 0.67, 95% CI 0.67-0.86) compared with control subjects without asthma and not receiving ICSs., Conclusions: BE+A was common and was associated with an increased risk of exacerbations and improved outcomes with ICS use. Unexpectedly we identified significantly lower mortality in patients with BE+A., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: A validated 4-point sputum colour chart can be used to objectively evaluate the levels of airway inflammation in bronchiectasis patients. In the European Bronchiectasis Registry (EMBARC), we tested whether sputum colour would be associated with disease severity and clinical outcomes., Methods: We used a prospective, observational registry of adults with bronchiectasis conducted in 31 countries. Patients who did not produce spontaneous sputum were excluded from the analysis. The Murray sputum colour chart was used at baseline and at follow-up visits. Key outcomes were frequency of exacerbations, hospitalisations for severe exacerbations and mortality during up to 5-year follow-up., Results: 13 484 patients were included in the analysis. More purulent sputum was associated with lower forced expiratory volume in 1 s (FEV 1 ), worse quality of life, greater bacterial infection and a higher bronchiectasis severity index. Sputum colour was strongly associated with the risk of future exacerbations during follow-up. Compared to patients with mucoid sputum (reference group), patients with mucopurulent sputum experienced significantly more exacerbations (incident rate ratio (IRR) 1.29, 95% CI 1.22-1.38; p<0.0001), while the rates were even higher for patients with purulent (IRR 1.55, 95% CI 1.44-1.67; p<0.0001) and severely purulent sputum (IRR 1.91, 95% CI 1.52-2.39; p<0.0001). Hospitalisations for severe exacerbations were also associated with increasing sputum colour with rate ratios, compared to patients with mucoid sputum, of 1.41 (95% CI 1.29-1.56; p<0.0001), 1.98 (95% CI 1.77-2.21; p<0.0001) and 3.05 (95% CI 2.25-4.14; p<0.0001) for mucopurulent, purulent and severely purulent sputum, respectively. Mortality was significantly increased with increasing sputum purulence, hazard ratio 1.12 (95% CI 1.01-1.24; p=0.027), for each increment in sputum purulence., Conclusion: Sputum colour is a simple marker of disease severity and future risk of exacerbations, severe exacerbations and mortality in patients with bronchiectasis., Competing Interests: Conflict of interest: S. Aliberti reports grants or contracts from Insmed Incorporated, Chiesi, Fisher & Paykel and GlaxoSmithKline, royalties or licences from McGraw Hill, consulting fees from Insmed Incorporated, Insmed Italy, Insmed Ireland Ltd, Zambon SpA, AstraZeneca UK Ltd, AstraZeneca Pharmaceutical LP, CSL Behring GmbH, Grifols, Fondazione Internazionale Menarini, Moderna, Chiesi, MCD Italia Srl, Brahms, PhysioAssist SAS and GlaxoSmithKline SpA, payment or honoraria for lectures, presentations, 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 Ltd and MSD Italia Srl. F.C. Ringshausen reports grants or contracts from the German Center for Lung Research (DZL), German Center for Infection Research (DZIF), IMI (EU/EFPIA), iABC Consortium (including Alaxia, Basilea, Novartis and Polyphor), Mukoviszidose Institute, Novartis, Insmed Germany, Grifols, Bayer and InfectoPharm, consulting fees from Parion, Grifols, Zambon, Insmed and Helmholtz-Zentrum für Infektionsforschung, payment or honoraria for lectures, presentations, manuscript writing or educational events from I!DE Werbeagentur GmbH, Interkongress GmbH, AstraZeneca, Insmed, Grifols and Universitätsklinikum Frankfurt am Main, payment for expert testimony from Social Court Cologne, support for attending meetings and/or travel from the German Kartagener Syndrome and Primary Ciliary Dyskinesia (PCD) Patient Advocacy Group Mukoviszidose eV, participation on a data safety monitoring board or advisory board for Insmed, Grifols and Shionogi, and leadership or fiduciary roles 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 Nontuberculous Mycobacterial Pulmonary Disease Registry EMBARC-NTM, Co-Speaker of the Medical Advisory Board of the German Kartagener Syndrome and PCD Patient Advocacy Group, Speaker of the Respiratory Infections and TB group of the German Respiratory Society (DGP), Speaker of the Cystic Fibrosis group of German Respiratory Society (DGP), PI of the German Center for Lung Research (DZL), Member of the Protocol Review Committee of the PCD-CTN, and Member of the Physician Association of the German Cystic Fibrosis Patient Advocacy Group; F.C. Ringshausen has other financial or non-financial interests with AstraZeneca, Boehringer Ingelheim, Celtaxsys, Corbus, Insmed, Novartis, Parion, University of Dundee, Vertex and Zambon. R. Dhar reports payment or honoraria for lectures, presentations, manuscript writing or educational events from LUPIN, CIPLA and Glenmark. C.S. Haworth reports payment or honoraria for lectures, presentations, manuscript writing or educational events from 30 Technology, CSL Behring, Chisi, Insmed, Janssen, LifeArc, Meiji, Mylan, Pneumagen, Shionogi, Vertex and Zambon. M.R. Loebinger reports consulting fees from Armata, 30T, AstraZeneca, Parion, Insmed, Chiesi, Zambon, Electromed, Recode, AN2 and Boehringer Ingelheim, payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed, and a leadership or fiduciary role as ERS Infection Group Chair. K. Dimakou reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Novartis, Boehringer Ingelheim, GlaxoSmithKline, Norma Hellas, Chiesi, AstraZeneca and Zambon, support for attending meetings and/or travel from Novartis, Boehringer Ingelheim, GlaxoSmithKline, Norma Hellas, Chiesi, AstraZeneca and Menarini, and participation on a data safety monitoring board or advisory board for Novartis, GlaxoSmithKline and Chiesi. M.L. Crichton reports consulting fees from Boxer Capital LLC. A. De Soyza reports grants or contracts from AstraZeneca, Pfizer, GlaxoSmithKline and Novartis, consulting fees from AstraZeneca, Insmed, GlaxoSmithKline, Boehringer, 30T and Bayer, and payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, Pfizer, GlaxoSmithKline and Novartis. M. Vendrell reports grants or contracts from Chiesi, payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed and Publi Creation, support for attending meetings and/or travel from Zambon, Chiesi, Novartis, Behring and Gebro, 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. P-R. Burgel reports grants or contracts from GlaxoSmithKline and Vertex, consulting fees from AstraZeneca, Chiesi, GlaxoSmithKline, Insmed, MSD, Vertex, Viatris and Zambon, and support for attending meetings and/or travel from AstraZeneca and Chiesi. S. Skrgat reports honoraria for educational events, invited lectures and presentations supported by Sanofi, AstraZeneca, Medis, Berlin-Chemie and Chiesi, and participation on a data safety monitoring board or advisory board for AstraZeneca. A. de Roux reports consulting fees from Adboard Insmed, payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed, AstraZeneca, GlaxoSmithKline, Berlin-Chemie and Boehringer Ingelheim, support for attending meetings and/or travel from AstraZeneca, and advisory board participation with Insmed. A. Bossios reports payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Chiesi, and leadership or fiduciary roles as Secretary of ERS Assembly 5 (Airway diseases, asthma, COPD and chronic cough), Vice-chair of the Nordic Severe Asthma Network (NSAN)-NORDSTAR and on the Steering Committee of ERS CRC Severe Asthma (SHARP). P. Kauppi reports support for attending meetings and/or travel from the Nordic Respiratory Academy, participation on a data safety monitoring board or advisory board for the Swedish Orphan Biovitrium, leadership or fiduciary roles for the Finnish Respiratory Society and Finnish Tuberculosis Foundation Grant Committee, and receipt of equipment, materials, drugs, medical writing, gifts or other services from Theravance. M. Murris reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Pfizer, payment for expert testimony from Vertex, support for attending meetings and/or travel from Zambon, and participation on a data safety monitoring board or advisory board for Zambon and Viatris. D. Obradovic reports a leadership or fiduciary role as president of the Serbian Society of Intensive Care Medicine. A. Amorim reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Zambon Group, and support for attending meetings and or/travel from Zambon Group, Boehringer Ingelheim and Novartis Farma. E. Van Braeckel reports grants or contracts from Insmed, Boehringer Ingelheim and Zambon. A. Shoemark reports consulting fees from Spirovant and Translate Bio, payment or honoraria for lectures, presentations, manuscript writing or educational events from Translate Bio, Ethris and Insmed, leadership or fiduciary roles in ERS CRCs (EMBARC, BEATPCD, AMR). M. Shteinberg reports consulting fees from GlaxoSmithKline, Boehringer Ingelheim, Kamada and Zambon, payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed, Boehringer Ingelheim, GlaxoSmithKline, AstraZeneca, Teva, Novartis, Kamada and Sanofi, support for attending meetings and/or travel from Novartis, Actelion, Boehringer Ingelheim, GlaxoSmithKline and Rafa, participation on a data safety monitoring board or advisory board for Bonus Therapeutics, Israel, leadership or fiduciary roles in EMBARC, Israel Pulmonology Society, and Israel Society for TB and Mycobacterial Diseases, receipt of equipment, materials, drugs, medical writing, gifts or other services from Trudell Medical International, and is Associate Editor of the American Journal of Respiratory and Critical Care Medicine. P.C. Goeminne reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Insmed, GlaxoSmithKline and Chiesi, support for attending meetings and/or travel from Chiesi, and participation on a data safety monitoring board or advisory board for Boehringer, GlaxoSmithKline and Pfizer. T. Welte reports grants from the German Ministry of Research and Education (BMBF), consulting fees and lecture honoraria from AstraZeneca, Chiesi, Grifols, GlaxoSmithKline, Insmed, MSD, Novartis, Pfizer and Sanofi, leadership roles for the German Center for Lung Research (DZL) – Board of Directors, Novartis Foundation – Board of Directors, Chairman of the Board of Trustees for CAPNETZ Foundation, German Lung Foundation, German Sepsis Foundation, and is Past President of the ERS, German Society of Pneumology and German Sepsis Society. F. Blasi reports grants or contracts from AstraZeneca, Chiesi, GlaxoSmithKline and Insmed, consulting fees from Menarini, GlaxoSmithKline and Om Pharma, and payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, Chiesi, GlaxoSmithKline, Guidotti, Grifols, Insmed, Menarini, Novartis, OM Pharma, Pfizer, Sanofi, Viatris, Vertex and Zambon. E. Polverino reports grants or contracts from Grifols, consulting fees from Insmed, Bayer, Chiesi and Zambon, payment or honoraria for lectures, presentations, manuscript writing or educational events from Bayer, Chiesi, Grifols, GlaxoSmithKline, Insmed, Menarini and Zambon, and support for attending meetings and/or travel from Insmed, Pfizer and Moderna. J.D. Chalmers reports grants or contracts from AstraZeneca, Boehringer Ingelheim, Genentech, Gilead Sciences, GlaxoSmithKline, Grifols, Insmed, LifeArc and Novartis, and consulting fees from AstraZeneca, Chiesi, GlaxoSmithKline, Insmed, Grifols, Novartis, Boehringer Ingelheim, Pfizer, Janssen, Antabio and Zambon, and is Chief Editor of the European Respiratory Journal. The remaining authors have no potential conflicts of interest to disclose., (Copyright ©The authors 2024.)

Gastro-oesophageal reflux disease (GORD) is a common comorbidity in bronchiectasis, and is often associated with poorer outcomes. The cause and effect relationship between GORD and bronchiectasis has not yet been fully elucidated and a greater understanding of the pathophysiology of the interaction and potential therapies is required. This review explores the underlying pathophysiology of GORD, its clinical presentation, risk factors, commonly applied diagnostic tools, and a detailed synthesis of original articles evaluating the prevalence of GORD, its influence on disease severity and current management strategies within the context of bronchiectasis. The prevalence of GORD in bronchiectasis ranges from 26% to 75%. Patients with co-existing bronchiectasis and GORD were found to have an increased mortality and increased bronchiectasis severity, manifest by increased symptoms, exacerbations, hospitalisations, radiological extent and chronic infection, with reduced pulmonary function and quality of life. The pathogenic role of Helicobacter pylori infection in bronchiectasis, perhaps via aspiration of gastric contents, also warrants further investigation. Our index of suspicion for GORD should remain high across the spectrum of disease severity in bronchiectasis. Identifying GORD in bronchiectasis patients may have important therapeutic and prognostic implications, although clinical trial evidence that treatment targeted at GORD can improve outcomes in bronchiectasis is currently lacking., (Copyright © 2018. Published by Elsevier Ltd.)

Introduction: Bronchiectasis is a multidimensional disease associated with substantial morbidity and mortality. Two disease-specific clinical prediction tools have been developed, the Bronchiectasis Severity Index (BSI) and the FACED score, both of which stratify patients into severity risk categories to predict the probability of mortality., Methods: We aimed to compare the predictive utility of BSI and FACED in assessing clinically relevant disease outcomes across seven European cohorts independent of their original validation studies., Results: The combined cohorts totalled 1612. Pooled analysis showed that both scores had a good discriminatory predictive value for mortality (pooled area under the curve (AUC) 0.76, 95% CI 0.74 to 0.78 for both scores) with the BSI demonstrating a higher sensitivity (65% vs 28%) but lower specificity (70% vs 93%) compared with the FACED score. Calibration analysis suggested that the BSI performed consistently well across all cohorts, while FACED consistently overestimated mortality in 'severe' patients (pooled OR 0.33 (0.23 to 0.48), p<0.0001). The BSI accurately predicted hospitalisations (pooled AUC 0.82, 95% CI 0.78 to 0.84), exacerbations, quality of life (QoL) and respiratory symptoms across all risk categories. FACED had poor discrimination for hospital admissions (pooled AUC 0.65, 95% CI 0.63 to 0.67) with low sensitivity at 16% and did not consistently predict future risk of exacerbations, QoL or respiratory symptoms. No association was observed with FACED and 6 min walk distance (6MWD) or lung function decline., Conclusion: The BSI accurately predicts mortality, hospital admissions, exacerbations, QoL, respiratory symptoms, 6MWD and lung function decline in bronchiectasis, providing a clinically relevant evaluation of disease severity., Competing Interests: Competing interests: None declared., (Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.)

Stress urinary incontinence (SUI) is frequently under-reported in patients with chronic lung disease and may have negative psychosocial consequences. We conducted a prospective study to determine the prevalence, severity and treatment outcomes of SUI in female bronchiectasis patients referred for airway clearance techniques. Nineteen out of 40 (48%) patients reported SUI symptoms. Of these, 14 (74%) reported a reduced quality of life secondary to SUI. Following personalised intervention, symptom improvement was observed in 13/19 (68%). Five out of 19 (26%) required specialist referral for further continence care. No associations with lung disease severity and SUI were noted. SUI is common in adult female bronchiectasis patients and should be routinely screened for to improve patients' overall quality of life.

Introduction: Idiopathic bronchiectasis is a poorly defined disease characterised by persistent inflammation, infection and progressive lung damage. Natural killer (NK) cells provide a major defense against infection, through the interaction of their surface receptors, including the activating and inhibitory killer immunoglobulin-like receptors (KIR), and human leukocyte antigens (HLA) class I molecules. Homozygosity for HLA-C has been shown in a single study to confer increased genetic susceptibility to idiopathic bronchiectasis. We aimed to assess whether the KIR and HLA repertoire, alone or in combination, may influence the risk of developing idiopathic bronchiectasis, in an independent replication study., Methods: In this prospective, observational, case-control association study, 79 idiopathic bronchiectasis patients diagnosed following extensive aetiological investigation were compared with 98 anonymous, healthy, age, sex and ethnically-matched controls attending blood donor sessions in the same geographical location. DNA extraction was performed according to standardised techniques. Determination of presence or absence of KIR genes was performed by a sequence specific oligonucleotide probe method. Allele frequencies for the proposed KIR, HLA-B and HLA-C risk alleles both individually and in combinations were compared., Results: We found no significant differences in allele frequency between the idiopathic bronchiectasis and control samples, whether considering HLA-C group homozygosity alone or in combination with the KIR type., Discussion: Our results do not show an association between HLA-C and KIR and therefore do not confirm previous positive findings. This may be explained by the lower frequency of HLA-C1 group homozygosity in the control population of the previous study (27.2%), compared to 42.3% in our study, which is consistent with the genetic profiling of control groups across the UK. The previous positive association study may therefore have been driven by an anomalous control group. Further larger prospective multicentre replication studies are needed to determine if an association exists., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Bronchiectasis is a chronic debilitating condition with considerable phenotypic diversity. A vicious cycle of infection and inflammation exists in damaged airways with patients suffering from persistent cough, purulent sputum production, recurrent chest infections and general malaise. The associated burden of disease in terms of increased morbidity, reduced quality of life and the socioeconomic cost of long-term management is significant. Further research is essential to improve our understanding of the development and progression of this disease. This article reviews what is currently known about bronchiectasis, its pathophysiology, aetiology and management strategies.

Background: Bronchiectasis is the outcome of a number of different airway insults. Very few studies have characterised the aetiology and utility of a dedicated screening proforma in adult patients attending a general bronchiectasis clinic., Methods: A prospective observational study of 189 bronchiectasis patients attending two centres in the North East of England over a two-year period was performed., Results: The aetiology of bronchiectasis was identified in 107/189(57%) patients. Idiopathic bronchiectasis (IB) represented the largest subgroup (43%). Post-infection bronchiectasis (PIB) constituted the largest proportion (24%) of known causes. Mean age (SD) at diagnosis was 54(20) years with a mean age at symptom onset of 37(24) years, accounting for a diagnostic delay of 17 years. Age of symptom onset was significantly younger in patients with PIB compared to IB (p < 0.0001) and in Pseudomonas sputum positive patients (p = 0.007). Screening for APBA and total immunoglobulin deficiency identified 9 (5%) patients who then had tailored treatment. Routine screening for other aetiologies was deemed unnecessary., Conclusion: IB and PIB accounted for two thirds of cases of bronchiectasis in a general population. We recommend routine screening for ABPA and total immunoglobulin deficiency but not for other rarer aetiologies., (Copyright © 2013 Elsevier Ltd. All rights reserved.)