Management erwachsener Patientinnen und Patienten mit Bronchiektasen-Erkrankung^[*]

Zusammenfassung

Die Bronchiektasen-Erkrankung ist eine ätiologisch heterogene, chronische und oftmals progredient verlaufende Atemwegs- und Lungenerkrankung, die durch eine irreversible Erweiterung der Bronchien gekennzeichnet ist. Sie geht häufig mit einer erheblichen Symptomlast, multiplen Komplikationen sowie einer eingeschränkten Lebensqualität einher. Seit mehreren Jahren ist weltweit eine deutliche Zunahme der Prävalenz der Bronchiektasen-Erkrankung mit einer relevanten ökonomischen Belastung der Gesundheitssysteme zu beobachten. Die vorliegende konsensusbasierte Leitlinie ist die erste deutschsprachige Leitlinie, die das Management der Bronchiektasen-Erkrankung bei Erwachsenen behandelt. Die Leitlinie betont die Wichtigkeit der thorakalen Bildgebung mittels CT zur Diagnose und Differenzierung der Bronchiektasen sowie die Bedeutung der Ätiologie zur Festlegung der Therapieansätze. Es werden sowohl nicht-medikamentöse als auch medikamentöse Therapien ausführlich erörtert. Zu den nicht-medikamentösen Maßnahmen gehören Raucherentwöhnung, Physiotherapie, körperliches Training, Rehabilitation, nichtinvasive Beatmung, Thoraxchirurgie und Lungentransplantation. Bei den medikamentösen Therapien wird besonders auf die langfristige Anwendung von Mukolytika, Bronchodilatatoren, antiinflammatorischen Medikamenten und Antibiotika eingegangen. Darüber hinaus geht die Leitlinie auf die Herausforderungen und Strategien bei der Behandlung einer oberen Atemwegsbeteiligung, von Komorbiditäten und Exazerbationen sowie die sozialmedizinischen Aspekte und das Schwerbehindertenrecht ein. Zudem wird die Bedeutung der Patientenaufklärung und des Selbstmanagements hervorgehoben. Abschließend werden spezielle Lebensphasen wie Transition, Kinderwunsch, Schwangerschaft und Elternschaft sowie Palliativmedizin behandelt. Die Leitlinie zielt darauf ab, eine umfassende, konsensusbasierte und patientenzentrierte Versorgung zu gewährleisten, wobei individuelle Risiken und Bedürfnisse berücksichtigt werden.

Abstract

Bronchiectasis is an etiologically heterogeneous, chronic, and often progressive respiratory disease characterized by irreversible bronchial dilation. It is frequently associated with significant symptom burden, multiple complications, and reduced quality of life. For several years, there has been a marked global increase in the prevalence of bronchiectasis, which is linked to a substantial economic burden on healthcare systems. This consensus-based guideline is the first German-language guideline addressing the management of bronchiectasis in adults. The guideline emphasizes the importance of thoracic imaging using CT for diagnosis and differentiation of bronchiectasis and highlights the significance of etiology in determining treatment approaches. Both non-drug and drug treatments are comprehensively covered. Non-pharmacological measures include smoking cessation, physiotherapy, physical training, rehabilitation, non-invasive ventilation, thoracic surgery, and lung transplantation. Pharmacological treatments focus on the long-term use of mucolytics, bronchodilators, anti-inflammatory medications, and antibiotics. Additionally, the guideline covers the challenges and strategies for managing upper airway involvement, comorbidities, and exacerbations, as well as socio-medical aspects and disability rights. The importance of patient education and self-management is also emphasized. Finally, the guideline addresses special life stages such as transition, family planning, pregnancy and parenthood, and palliative care. The aim is to ensure comprehensive, consensus-based, and patient-centered care, taking into account individual risks and needs.

^* Verabschiedet von den Vorständen der beteiligten Fachgesellschaften und Organisationen am 03.04.2024

^# Geteilte Letztautorenschaft

Publication History

Article published online:
08 November 2024

• Literatur

• 1 Aliberti S, Goeminne PC, OʼDonnell AE. et al. Criteria and definitions for the radiological and clinical diagnosis of bronchiectasis in adults for use in clinical trials: international consensus recommendations. Lancet Respir Med 2022; 10: 298-306
• 2 Flume PA, Chalmers JD, Olivier KN. Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity. Lancet 2018; 392: 880-890
• 3 Chandrasekaran R, Mac Aogain M, Chalmers JD. et al. Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis. BMC Pulm Med 2018; 18: 83
• 4 Chalmers JD, Polverino E, Crichton ML. et al. Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC). Lancet Respir Med 2023; 11: 637-649
• 5 Keir HR, Chalmers JD. Pathophysiology of Bronchiectasis. Semin Respir Crit Care Med 2021; 42: 499-512
• 6 Seitz AE, Olivier KN, Steiner CA. et al. Trends and burden of bronchiectasis-associated hospitalizations in the United States, 1993-2006. Chest 2010; 138: 944-949
• 7 Seitz AE, Olivier KN, Adjemian J. et al. Trends in Bronchiectasis among Medicare Beneficiaries in the United States, 2000–2007. Chest 2012; 142: 432-439
• 8 Ringshausen FC, Roux A de, Pletz MW. et al. Bronchiectasis-associated hospitalizations in Germany, 2005–2011: a population-based study of disease burden and trends. PLoS One 2013; 8: e71109
• 9 Quint JK, Millett ER, Joshi M. et al. Changes in the incidence, prevalence and mortality of bronchiectasis in the UK from 2004 to 2013: a population-based cohort study. Eur Respir J 2016; 47: 186-193
• 10 Sanchez-Munoz G, Lopez de Andres A, Jimenez-Garcia R. et al. Time Trends in Hospital Admissions for Bronchiectasis: Analysis of the Spanish National Hospital Discharge Data (2004 to 2013). PLoS One 2016; 11: e0162282
• 11 Ringshausen FC, Rademacher J, Pink I. et al. Increasing bronchiectasis prevalence in Germany, 2009–2017: a population-based cohort study. Eur Respir J 2019; 54: 1900499
• 12 Ringshausen FC, Roux A de, Diel R. et al. Bronchiectasis in Germany: a population-based estimation of disease prevalence. Eur Respir J 2015; 46: 1805-1807
• 13 Monteagudo M, Rodriguez-Blanco T, Barrecheguren M. et al. Prevalence and incidence of bronchiectasis in Catalonia, Spain: A population-based study. Respir Med 2016; 121: 26-31
• 14 Chang AB, Grimwood K, Mulholland EK. et al. Bronchiectasis in indigenous children in remote Australian communities. Med J Aust 2002; 177: 200-204
• 15 Bibby S, Milne R, Beasley R. Hospital admissions for non-cystic fibrosis bronchiectasis in New Zealand. N Z Med J 2015; 128: 30-38
• 16 Quellhorst L, Barten-Neiner G, Roux A de. et al. Psychometric Validation of the German Translation of the Quality of Life Questionnaire-Bronchiectasis (QOL-B)-Data from the German Bronchiectasis Registry PROGNOSIS. J Clin Med 2022; 11: 441
• 17 Aliberti S, Polverino E, Chalmers JD. et al. The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) ERS Clinical Research Collaboration. Eur Respir J 2018; 52: 1802074
• 18 Joish VN, Spilsbury-Cantalupo M, Operschall E. et al. Economic burden of non-cystic fibrosis bronchiectasis in the first year after diagnosis from a US health plan perspective. Appl Health Econ Health Policy 2013; 11: 299-304
• 19 Diel R, Chalmers JD, Rabe KF. et al. Economic burden of bronchiectasis in Germany. Eur Respir J 2019; 53: 1802033
• 20 Rosa Carrillo D de la, Navarro Rolon A, Giron Moreno RM. et al. Cost of Hospitalizations due to Exacerbation in Patients with Non-Cystic Fibrosis Bronchiectasis. Respiration 2018; 96: 406-416
• 21 Goyal V, McPhail SM, Hurley F. et al. Cost of hospitalization for bronchiectasis exacerbation in children. Respirology 2020; 25: 1250-1256
• 22 Roberts JM, Goyal V, Kularatna S. et al. The Economic Burden of Bronchiectasis: A Systematic Review. Chest 2023; 164: 1396-1421
• 23 Bruggen-Bogaarts BA van der, Bruggen HM vn der, Waes PF van. et al. Screening for bronchiectasis. A comparative study between chest radiography and high-resolution CT. Chest 1996; 109: 608-611
• 24 Young K, Aspestrand F, Kolbenstvedt A. High resolution CT and bronchography in the assessment of bronchiectasis. Acta Radiol 1991; 32: 439-441
• 25 Jung KJ, Lee KS, Kim SY. et al. Low-dose, volumetric helical CT: image quality, radiation dose, and usefulness for evaluation of bronchiectasis. Invest Radiol 2000; 35: 557-563
• 26 Biederer J, Beer M, Hirsch W. et al. MRI of the lung (2/3). Why … when … how?. Insights Imaging 2012; 3: 355-371
• 27 Wielputz MO, Eichinger M, Biederer J. et al. Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation. Rofo 2016; 188: 834-845
• 28 Woods JC, Wild JM, Wielputz MO. et al. Current state of the art MRI for the longitudinal assessment of cystic fibrosis. J Magn Reson Imaging 2020; 52: 1306-1320
• 29 Bauman G, Puderbach M, Heimann T. et al. Validation of Fourier decomposition MRI with dynamic contrast-enhanced MRI using visual and automated scoring of pulmonary perfusion in young cystic fibrosis patients. Eur J Radiol 2013; 82: 2371-2377
• 30 Voskrebenzev A, Gutberlet M, Klimes F. et al. Feasibility of quantitative regional ventilation and perfusion mapping with phase-resolved functional lung (PREFUL) MRI in healthy volunteers and COPD, CTEPH, and CF patients. Magn Reson Med 2018; 79: 2306-2314
• 31 Meerburg JJ, Veerman GDM, Aliberti S. et al. Diagnosis and quantification of bronchiectasis using computed tomography or magnetic resonance imaging: A systematic review. Respir Med 2020; 170: 105954
• 32 Bankier AA, MacMahon H, Colby T. et al. Fleischner Society: Glossary of Terms for Thoracic Imaging. Radiology 2024; 310: e232558
• 33 Bhalla M, Turcios N, Aponte V. et al. Cystic fibrosis: scoring system with thin-section CT. Radiology 1991; 179: 783-788
• 34 Reiff DB, Wells AU, Carr DH. et al. CT findings in bronchiectasis: limited value in distinguishing between idiopathic and specific types. AJR Am J Roentgenol 1995; 165: 261-267
• 35 Brody AS, Klein JS, Molina PL. et al. High-resolution computed tomography in young patients with cystic fibrosis: distribution of abnormalities and correlation with pulmonary function tests. J Pediatr 2004; 145: 32-38
• 36 Bedi P, Chalmers JD, Goeminne PC. et al. The BRICS (Bronchiectasis Radiologically Indexed CT Score): A Multicenter Study Score for Use in Idiopathic and Postinfective Bronchiectasis. Chest 2018; 153: 1177-1186
• 37 Konietzke P, Weinheimer O, Wielputz MO. et al. Quantitative CT detects changes in airway dimensions and air-trapping after bronchial thermoplasty for severe asthma. Eur J Radiol 2018; 107: 33-38
• 38 Konietzke P, Wielputz MO, Wagner WL. et al. Quantitative CT detects progression in COPD patients with severe emphysema in a 3-month interval. Eur Radiol 2020; 30: 2502-2512
• 39 Tiddens H, Meerburg JJ, Eerden MM van der. et al. The radiological diagnosis of bronchiectasis: whatʼs in a name?. Eur Respir Rev 2020; 29
• 40 Chalmers JD, Elborn JS. Reclaiming the name ‘bronchiectasis’. Thorax 2015; 70: 399-400
• 41 Ringshausen FC, Hellmuth T, Dittrich AM. [Evidence-based treatment of cystic fibrosis]. Internist (Berl) 2020; 61: 1212-1229
• 42 Winter DH, Manzini M, Salge JM. et al. Aging of the lungs in asymptomatic lifelong nonsmokers: findings on HRCT. Lung 2015; 193: 283-290
• 43 Polverino E, Dimakou K, Hurst J. et al. The overlap between bronchiectasis and chronic airway diseases: state of the art and future directions. Eur Respir J 2018; 52: 1800328
• 44 Hill AT, Haworth CS, Aliberti S. et al. Pulmonary exacerbation in adults with bronchiectasis: a consensus definition for clinical research. Eur Respir J 2017; 49: 1700051
• 45 Perl S, Shteinberg M. Bronchiectasis Exacerbations: Definitions, Causes, and Acute Management. Semin Respir Crit Care Med 2021; 42: 595-605
• 46 Park YE, Sung H, Oh YM. Respiratory Viruses in Acute Exacerbations of Bronchiectasis. J Korean Med Sci 2021; 36: e217
• 47 Chen CL, Huang Y, Yuan JJ. et al. The Roles of Bacteria and Viruses in Bronchiectasis Exacerbation: A Prospective Study. Arch Bronconeumol (Engl Ed) 2020; 56: 621-629
• 48 Gao YH, Guan WJ, Xu G. et al. The role of viral infection in pulmonary exacerbations of bronchiectasis in adults: a prospective study. Chest 2015; 147: 1635-1643
• 49 Mitchell AB, Mourad B, Buddle L. et al. Viruses in bronchiectasis: a pilot study to explore the presence of community acquired respiratory viruses in stable patients and during acute exacerbations. BMC Pulm Med 2018; 18: 84
• 50 Crichton ML, Shoemark A, Chalmers JD. The Impact of the COVID-19 Pandemic on Exacerbations and Symptoms in Bronchiectasis: A Prospective Study. Am J Respir Crit Care Med 2021; 204: 857-859
• 51 Goeminne PC, Cox B, Finch S. et al. The impact of acute air pollution fluctuations on bronchiectasis pulmonary exacerbation: a case-crossover analysis. Eur Respir J 2018; 52: 1702557
• 52 Mac Aogain M, Narayana JK, Tiew PY. et al. Integrative microbiomics in bronchiectasis exacerbations. Nat Med 2021; 27: 688-699
• 53 Chalmers JD, Aliberti S, Filonenko A. et al. Characterization of the “Frequent Exacerbator Phenotype” in Bronchiectasis. Am J Respir Crit Care Med 2018; 197: 1410-1420
• 54 Chalmers JD, Moffitt KL, Suarez-Cuartin G. et al. Neutrophil Elastase Activity Is Associated with Exacerbations and Lung Function Decline in Bronchiectasis. Am J Respir Crit Care Med 2017; 195: 1384-1393
• 55 Chalmers JD, Goeminne P, Aliberti S. et al. The bronchiectasis severity index. An international derivation and validation study. Am J Respir Crit Care Med 2014; 189: 576-585
• 56 Abo-Leyah H, Finch S, Keir H. et al. Peripheral blood eosinophilia and clinical phenotype in Bronchiectasis. Eur Respir J 2018; 52: PA2665
• 57 Shoemark A, Shteinberg M, De Soyza A. et al. Characterization of Eosinophilic Bronchiectasis: A European Multicohort Study. Am J Respir Crit Care Med 2022; 205: 894-902
• 58 Polverino E, Dimakou K, Traversi L. et al. Bronchiectasis and asthma: Data from The European Bronchiectasis Registry (EMBARC). J Allergy Clin Immunol 2024;
• 59 Lucas JS, Gahleitner F, Amorim A. et al. Pulmonary exacerbations in patients with primary ciliary dyskinesia: an expert consensus definition for use in clinical trials. ERJ Open Res 2019; 5: 00147-2018
• 60 Polverino E, Goeminne PC, McDonnell MJ. et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J 2017; 50: 1700629
• 61 Hill AT, Sullivan AL, Chalmers JD. et al. British Thoracic Society guideline for bronchiectasis in adults. BMJ Open Respir Res 2018; 5: e000348
• 62 Martinez-Garcia MA, Maiz L, Olveira C. et al. Spanish Guidelines on Treatment of Bronchiectasis in Adults. Arch Bronconeumol (Engl Ed) 2018; 54: 88-98
• 63 Brill SE, Patel AR, Singh R. et al. Lung function, symptoms and inflammation during exacerbations of non-cystic fibrosis bronchiectasis: a prospective observational cohort study. Respir Res 2015; 16: 16
• 64 Menendez R, Mendez R, Amara-Elori I. et al. Systemic Inflammation during and after Bronchiectasis Exacerbations: Impact of Pseudomonas aeruginosa. J Clin Med 2020; 9: 2631
• 65 Murray MP, Turnbull K, Macquarrie S. et al. Assessing response to treatment of exacerbations of bronchiectasis in adults. Eur Respir J 2009; 33: 312-318
• 66 Mammadov F, Olgun Yildizeli S, Kocakaya D. et al. The role of procalcitonin as a biomarker for acute pulmonary exacerbation in subjects with cystic fibrosis and non-cystic fibrosis bronchiectasis. Marmara Med J 2022; 35: 164-171
• 67 Martinez-Garcia MA, Athanazio RA, Giron R. et al. Predicting high risk of exacerbations in bronchiectasis: the E-FACED score. Int J Chron Obstruct Pulmon Dis 2017; 12: 275-284
• 68 Araujo D, Shteinberg M, Aliberti S. et al. The independent contribution of Pseudomonas aeruginosa infection to long-term clinical outcomes in bronchiectasis. Eur Respir J 2018; 51:  1701953
• 69 Finch S, McDonnell MJ, Abo-Leyah H. et al. A Comprehensive Analysis of the Impact of Pseudomonas aeruginosa Colonization on Prognosis in Adult Bronchiectasis. Annals of the American Thoracic Society 2015; 12: 1602-1611
• 70 Schwarz C, Bend J, Hebestreit H. et al. [CF Lung Disease – a German S3 Guideline: Pseudomonas aeruginosa]. Pneumologie 2024;
• 71 Laennec RTH. De l’Auscultation Médiate ou Traité du Diagnostic des Maladies des Poumons et du Coeur. Paris: Brosson & Chaudé; 1819
• 72 King PT, Daviskas E. Pathogenesis and diagnosis of bronchiectasis. Breathe 2010; 6: 342-351
• 73 Chalmers JD, Chang AB, Chotirmall SH. et al. Bronchiectasis. Nat Rev Dis Primers 2018; 4: 45
• 74 Cole PJ. Inflammation: a two-edged sword--the model of bronchiectasis. Eur J Respir Dis Suppl 1986; 147: 6-15
• 75 Eastham KM, Fall AJ, Mitchell L. et al. The need to redefine non-cystic fibrosis bronchiectasis in childhood. Thorax 2004; 59: 324-327
• 76 Eden E, Choate R, Barker A. et al. The Clinical Features of Bronchiectasis Associated with Alpha-1 Antitrypsin Deficiency, Common Variable Immunodeficiency and Primary Ciliary Dyskinesia – Results from the U.S. Bronchiectasis Research Registry. Chronic Obstr Pulm Dis 2019; 6: 145-153
• 77 Bush A, Floto RA. Pathophysiology, causes and genetics of paediatric and adult bronchiectasis. Respirology 2019; 24: 1053-1062
• 78 Carpagnano GE, Scioscia G, Lacedonia D. et al. Severe uncontrolled asthma with bronchiectasis: a pilot study of an emerging phenotype that responds to mepolizumab. J Asthma Allergy 2019; 12: 83-90
• 79 Rademacher J, Konwert S, Fuge J. et al. Anti-IL5 and anti-IL5Ralpha therapy for clinically significant bronchiectasis with eosinophilic endotype: a case series. Eur Respir J 2020; 55: 1901333
• 80 Aliberti S, Sotgiu G, Blasi F. et al. Blood eosinophils predict inhaled fluticasone response in bronchiectasis. European Respiratory Journal 2020; 56: 2000453
• 81 Khan T, Jose RJ, Renzoni EA. et al. A Closer Look at the Role of Anti-CCP Antibodies in the Pathogenesis of Rheumatoid Arthritis-Associated Interstitial Lung Disease and Bronchiectasis. Rheumatol Ther 2021; 8: 1463-1475
• 82 Lhote R, Theodore C, Issoufaly T. et al. Successful treatment of antineutrophil cytoplasmic antibody-associated bronchiectasis with immunosuppressive therapy. Eur Respir J 2015; 46: 554-557
• 83 Lhote R, Chilles M, Groh M. et al. Spectrum and Prognosis of Antineutrophil Cytoplasmic Antibody-associated Vasculitis-related Bronchiectasis: Data from 61 Patients. J Rheumatol 2020; 47: 1522-1531
• 84 Rademacher J, Mertsch P, Roux A de. et al. The german bronchiectasis registry (PROGNOSIS): results from 1000 patients. Pneumologie 2019; 73: V717
• 85 Morice AH, Millqvist E, Bieksiene K. et al. ERS guidelines on the diagnosis and treatment of chronic cough in adults and children. Eur Respir J 2020; 55: 1901136
• 86 Chang AB, Fortescue R, Grimwood K. et al. European Respiratory Society guidelines for the management of children and adolescents with bronchiectasis. Eur Respir J 2021; 58: 2002990
• 87 Boaventura R, Sibila O, Agusti A. et al. Treatable traits in bronchiectasis. Eur Respir J 2018; 52: 1801269
• 88 Chalmers JD, Chotirmall SH. Bronchiectasis: new therapies and new perspectives. Lancet Respir Med 2018; 6: 715-726
• 89 Rademacher J, Roux A de, Ringshausen FC. [PROGNOSIS – The PROspective German NOn-CF BronchiectaSIS Patient Registry]. Pneumologie 2015; 69: 391-393
• 90 Graeber SY, Renz DM, Stahl M. et al. Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on Lung Clearance Index and Magnetic Resonance Imaging in Patients with Cystic Fibrosis and One or Two F508del Alleles. Am J Respir Crit Care Med 2022; 206: 311-320
• 91 Graeber SY, Vitzthum C, Pallenberg ST. et al. Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles. Am J Respir Crit Care Med 2022; 205: 540-549
• 92 Ringshausen FC, Sauer-Heilborn A, Buttner T. et al. Lung transplantation for end-stage cystic fibrosis before and after the availability of elexacaftor-tezacaftor-ivacaftor, Germany, 2012–2021. Eur Respir J 2023; 61: 2201402
• 93 Daines CL, Tullis E, Costa S. et al. Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study. Eur Respir J 2023; 62: 2202029
• 94 Araujo D, Shteinberg M, Aliberti S. et al. Standardised classification of the aetiology of bronchiectasis using an objective algorithm. Eur Respir J 2017; 50: 1701289
• 95 Shoemark A, Ozerovitch L, Wilson R. Aetiology in adult patients with bronchiectasis. Respir Med 2007; 101: 1163-1170
• 96 OʼDonnell AE. Bronchiectasis update. Curr Opin Infect Dis 2018; 31: 194-198
• 97 Chalmers JD, Ringshausen FC, Harris B. et al. Cross-infection risk in patients with bronchiectasis: a position statement from the European Bronchiectasis Network (EMBARC), EMBARC/ELF patient advisory group and European Reference Network (ERN-Lung) Bronchiectasis Network. Eur Respir J 2018; 51: 1701937
• 98 Hogardt M, Kahl B, Besier S. et al. Atemwegsinfektionen bei Mukoviszidose. 2. Aufl.. München, Jena: Urban & Fischer Verlag/Elsevier GmbH; 2019
• 99 Bundesärztekammer. Richtlinie der Bundesärztekammer zur Qualitätssicherung laboratoriumsmedizinischer Untersuchungen (Rili-BÄK). Dtsch Arztebl 2023; 120: A-994/B-858
• 100 Proctor RA, Eiff C von, Kahl BC. et al. Small colony variants: a pathogenic form of bacteria that facilitates persistent and recurrent infections. Nat Rev Microbiol 2006; 4: 295-305
• 101 Haworth CS, Banks J, Capstick T. et al. British Thoracic Society guidelines for the management of non-tuberculous mycobacterial pulmonary disease (NTM-PD). Thorax 2017; 72: ii1-ii64
• 102 Hill AT, Pasteur M, Cornford C. et al. Primary care summary of the British Thoracic Society Guideline on the management of non-cystic fibrosis bronchiectasis. Prim Care Respir J 2011; 20: 135-140
• 103 Aksamit TR, OʼDonnell AE, Barker A. et al. Adult Patients With Bronchiectasis: A First Look at the US Bronchiectasis Research Registry. Chest 2017; 151: 982-992
• 104 Choate R, Aksamit TR, Mannino D. et al. Pseudomonas aeruginosa associated with severity of non-cystic fibrosis bronchiectasis measured by the modified bronchiectasis severity score (BSI) and the FACED: The US bronchiectasis and NTM Research Registry (BRR) study. Respir Med 2021; 177: 106285
• 105 Marra R, Sgalla G, Richeldi L. et al. Role of Stenotrophomonas maltophilia isolation in patients with non-CF bronchiectasis. QJM 2020; 113: 726-730
• 106 Metersky ML, Choate R, Aksamit TR. et al. Stenotrophomonas maltophilia in patients with bronchiectasis: An analysis of the US bronchiectasis and NTM Research Registry. Respir Med 2022; 193: 106746
• 107 Dicker AJ, Lonergan M, Keir HR. et al. The sputum microbiome and clinical outcomes in patients with bronchiectasis: a prospective observational study. Lancet Respir Med 2021; 9: 885-896
• 108 Martinez-Garcia MA, Gracia J de, Vendrell Relat M. et al. Multidimensional approach to non-cystic fibrosis bronchiectasis: the FACED score. Eur Respir J 2014; 43: 1357-1367
• 109 Blanchette CM, Noone JM, Stone G. et al. Healthcare Cost and Utilization before and after Diagnosis of Pseudomonas aeruginosa among Patients with Non-Cystic Fibrosis Bronchiectasis in the U.S. Med Sci (Basel) 2017; 5: 20
• 110 Goeminne PC, Hernandez F, Diel R. et al. The economic burden of bronchiectasis – known and unknown: a systematic review. BMC Pulm Med 2019; 19: 54
• 111 Zhu YN, Xie JQ, He XW. et al. Prevalence and Clinical Characteristics of Nontuberculous Mycobacteria in Patients with Bronchiectasis: A Systematic Review and Meta-Analysis. Respiration 2021; 100: 1218-1229
• 112 Ingen J van, Bendien SA, Lange WC de. et al. Clinical relevance of non-tuberculous mycobacteria isolated in the Nijmegen-Arnhem region, The Netherlands. Thorax 2009; 64: 502-506
• 113 Jankovic M, Sabol I, Zmak L. et al. Microbiological criteria in non-tuberculous mycobacteria pulmonary disease: a tool for diagnosis and epidemiology. Int J Tuberc Lung Dis 2016; 20: 934-940
• 114 Gommans EP, Even P, Linssen CF. et al. Risk factors for mortality in patients with pulmonary infections with non-tuberculous mycobacteria: a retrospective cohort study. Respir Med 2015; 109: 137-145
• 115 Wong C, Jayaram L, Karalus N. et al. Azithromycin for prevention of exacerbations in non-cystic fibrosis bronchiectasis (EMBRACE): a randomised, double-blind, placebo-controlled trial. Lancet 2012; 380: 660-667
• 116 Ji B, Lounis N, Truffot-Pernot C. et al. Selection of resistant mutants of Mycobacterium avium in beige mice by clarithromycin monotherapy. Antimicrob Agents Chemother 1992; 36: 2839-2840
• 117 Daley CL, Iaccarino JM, Lange C. et al. Treatment of nontuberculous mycobacterial pulmonary disease: an official ATS/ERS/ESCMID/IDSA clinical practice guideline. Eur Respir J 2020; 56: 2000535
• 118 Maiz L, Vendrell M, Olveira C. et al. Prevalence and factors associated with isolation of Aspergillus and Candida from sputum in patients with non-cystic fibrosis bronchiectasis. Respiration 2015; 89: 396-403
• 119 Mac Aogáin M, Chandrasekaran R, Lim AYH. et al. Immunological corollary of the pulmonary mycobiome in bronchiectasis: the CAMEB study. Eur Respir J 2018; 52: 1800766
• 120 Yang B, Kim T, Ryu J. et al. Increased Incidence and Associated Risk Factors of Aspergillosis in Patients with Bronchiectasis. J Pers Med 2021; 11: 422
• 121 Chrdle A, Mustakim S, Bright-Thomas RJ. et al. Aspergillus bronchitis without significant immunocompromise. Ann N Y Acad Sci 2012; 1272: 73-85
• 122 Salzer HJ, Heyckendorf J, Kalsdorf B. et al. Characterization of patients with chronic pulmonary aspergillosis according to the new ESCMID/ERS/ECMM and IDSA guidelines. Mycoses 2017; 60: 136-142
• 123 Chotirmall SH, Martin-Gomez MT. Aspergillus Species in Bronchiectasis: Challenges in the Cystic Fibrosis and Non-cystic Fibrosis Airways. Mycopathologia 2018; 183: 45-59
• 124 Aaron SD, Vandemheen KL, Freitag A. et al. Treatment of Aspergillus fumigatus in patients with cystic fibrosis: a randomized, placebo-controlled pilot study. PLoS One 2012; 7: e36077
• 125 Denning DW, Cadranel J, Beigelman-Aubry C. et al. Chronic pulmonary aspergillosis: rationale and clinical guidelines for diagnosis and management. Eur Respir J 2016; 47: 45-68
• 126 Maitre T, Cottenet J, Godet C. et al. Chronic pulmonary aspergillosis: prevalence, favouring pulmonary diseases and prognosis. Eur Respir J 2021; 58: 2003345
• 127 Phoompoung P, Chayakulkeeree M. Chronic Pulmonary Aspergillosis Following Nontuberculous Mycobacterial Infections: An Emerging Disease. J Fungi (Basel) 2020; 6: 346
• 128 Smith NL, Denning DW. Underlying conditions in chronic pulmonary aspergillosis including simple aspergilloma. European Respiratory Journal 2011; 37: 865-872
• 129 Jhun BW, Jeon K, Eom JS. et al. Clinical characteristics and treatment outcomes of chronic pulmonary aspergillosis. Med Mycol 2013; 51: 811-817
• 130 Kunst H, Wickremasinghe M, Wells A. et al. Nontuberculous mycobacterial disease and Aspergillus-related lung disease in bronchiectasis. European Respiratory Journal 2006; 28: 352-357
• 131 Denning DW, Pleuvry A, Cole DC. Global burden of chronic pulmonary aspergillosis as a sequel to pulmonary tuberculosis. B World Health Organ 2011; 89: 864-872
• 132 Page ID, Byanyima R, Hosmane S. et al. Chronic pulmonary aspergillosis commonly complicates treated pulmonary tuberculosis with residual cavitation. Eur Respir J 2019; 53: 1801184
• 133 Lowes D, Al-Shair K, Newton PJ. et al. Predictors of mortality in chronic pulmonary aspergillosis. Eur Respir J 2017; 49: 1601062
• 134 Zoumot Z, Boutou AK, Gill SS. et al. Mycobacterium avium complex infection in non-cystic fibrosis bronchiectasis. Respirology 2014; 19: 714-722
• 135 Fukushima K, Kitada S, Abe Y. et al. Long-Term Treatment Outcome of Progressive Mycobacterium avium Complex Pulmonary Disease. Journal of Clinical Medicine 2020; 9: 1315
• 136 Takeda K, Imamura Y, Takazono T. et al. The risk factors for developing of chronic pulmonary aspergillosis in nontuberculous mycobacteria patients and clinical characteristics and outcomes in chronic pulmonary aspergillosis patients coinfected with nontuberculous mycobacteria. Med Mycol 2016; 54: 120-127
• 137 Alastruey-Izquierdo A, Cadranel J, Flick H. et al. Treatment of Chronic Pulmonary Aspergillosis: Current Standards and Future Perspectives. Respiration 2018; 96: 159-170
• 138 Ullmann AJ, Aguado JM, Arikan-Akdagli S. et al. Diagnosis and management of Aspergillus diseases: executive summary of the 2017 ESCMID-ECMM-ERS guideline. Clin Microbiol Infect 2018; 24 (Suppl. 01) e1-e38
• 139 Mitchelmore PJ, Randall J, Bull MJ. et al. Molecular epidemiology of Pseudomonas aeruginosa in an unsegregated bronchiectasis cohort sharing hospital facilities with a cystic fibrosis cohort. Thorax 2018; 73: 677-679
• 140 De Soyza A, Perry A, Hall AJ. et al. Molecular epidemiological analysis suggests cross-infection with Pseudomonas aeruginosa is rare in non-cystic fibrosis bronchiectasis. Eur Respir J 2014; 43: 900-903
• 141 Hilliam Y, Moore MP, Lamont IL. et al. Pseudomonas aeruginosa adaptation and diversification in the non-cystic fibrosis bronchiectasis lung. Eur Respir J 2017; 49: 1602108
• 142 Woo TE, Lim R, Surette MG. et al. Epidemiology and natural history of Pseudomonas aeruginosa airway infections in non-cystic fibrosis bronchiectasis. ERJ Open Res 2018; 4: 00162-2017
• 143 Cramer N, Sedlacek L, Tummler B. et al. Low transmission risk of Pseudomonas aeruginosa in a bronchiectasis clinic based on the knowledge of bacterial population biology. Eur Respir J 2019; 53: 1802191
• 144 Rosenboom I, Oguz S, Ludemann IM. et al. Pseudomonas aeruginosa population genomics among adults with bronchiectasis across Germany. ERJ Open Res 2023; 9: 00156-2023
• 145 Southern KW, Burgel PR, Castellani C. et al. Standards for the care of people with cystic fibrosis (CF). J Cyst Fibros 2023; 22: 961-962
• 146 Hurst JR, Cumella A, Niklewicz CN. et al. Acceptability of hygiene, face covering and social distancing interventions to prevent exacerbations in people living with airways diseases. Thorax 2022; 77: 505-507
• 147 Bell J, Alexander L, Carson J. et al. Nebuliser hygiene in cystic fibrosis: evidence-based recommendations. Breathe (Sheff) 2020; 16: 190328
• 148 Müller F, Bend J, Huttegger I. et al. S3-Leitlinie „Lungenerkrankung bei Mukoviszidose“ Modul 1: Diagnostik und Therapie nach dem ersten Nachweis von Pseudomonas aeruginosa. Monatsschr Kinderheilkd 2015; 6: 590-599
• 149 Langton Hewer SC, Smith S, Rowbotham NJ. et al. Antibiotic strategies for eradicating Pseudomonas aeruginosa in people with cystic fibrosis. Cochrane Database Syst Rev 2023; 6: CD004197
• 150 Mogayzel J5r PJ, Naureckas ET, Robinson KA. et al. Cystic Fibrosis Foundation pulmonary guideline. pharmacologic approaches to prevention and eradication of initial Pseudomonas aeruginosa infection. Ann Am Thorac Soc 2014; 11: 1640-1650
• 151 Emerson J, Rosenfeld M, McNamara S. et al. Pseudomonas aeruginosa and other predictors of mortality and morbidity in young children with cystic fibrosis. Pediatr Pulmonol 2002; 34: 91-100
• 152 Pamukcu A, Bush A, Buchdahl R. Effects of pseudomonas aeruginosa colonization on lung function and anthropometric variables in children with cystic fibrosis. Pediatr Pulmonol 1995; 19: 10-15
• 153 White L, Mirrani G, Grover M. et al. Outcomes of Pseudomonas eradication therapy in patients with non-cystic fibrosis bronchiectasis. Respir Med 2012; 106: 356-360
• 154 Vallieres E, Tumelty K, Tunney MM. et al. Efficacy of Pseudomonas aeruginosa eradication regimens in bronchiectasis. Eur Respir J 2017; 49: 1600851
• 155 Pieters A, Bakker M, Hoek RAS. et al. The clinical impact of Pseudomonas aeruginosa eradication in bronchiectasis in a Dutch referral centre. Eur Respir J 2019; 53: 1802081
• 156 Orriols R, Hernando R, Ferrer A. et al. Eradication Therapy against Pseudomonas aeruginosa in Non-Cystic Fibrosis Bronchiectasis. Respiration 2015; 90: 299-305
• 157 Blanco-Aparicio M, Saleta Canosa JL, Valino Lopez P. et al. Eradication of Pseudomonas aeruginosa with inhaled colistin in adults with non-cystic fibrosis bronchiectasis. Chron Respir Dis 2019; 16: 1479973119872513
• 158 Conceição M, Shteinberg M, Goeminne P. et al. Eradication treatment for Pseudomonas aeruginosa infection in adults with bronchiectasis: a systematic review and meta-analysis. Eur Respir Rev 2024; 33: 230178
• 159 Suarez-Cuartin G, Smith A, Abo-Leyah H. et al. Anti-Pseudomonas aeruginosa IgG antibodies and chronic airway infection in bronchiectasis. Respir Med 2017; 128: 1-6
• 160 Kappler M, Nagel F, Feilcke M. et al. Predictive values of antibodies against Pseudomonas aeruginosa in patients with cystic fibrosis one year after early eradication treatment. J Cyst Fibros 2014; 13: 534-541
• 161 Mauch RM, Levy CE. Serum antibodies to Pseudomonas aeruginosa in cystic fibrosis as a diagnostic tool: a systematic review. J Cyst Fibros 2014; 13: 499-507
• 162 Drain M, Elborn JS. Assessment and investigation of adults with bronchiectasis. Floto RA, Haworth CS. eds. In: European Respiratory Monograph Vol 52 (Bronchiectasis). Sheffield: European Respiratory Society; 2011: 32-43
• 163 Pasteur MC, Bilton D, Hill AT. et al. British Thoracic Society guideline for non-CF bronchiectasis. Thorax 2010; 65 (Suppl. 01) i1-58
• 164 Lonni S, Chalmers JD, Goeminne PC. et al. Etiology of Non-Cystic Fibrosis Bronchiectasis in Adults and Its Correlation to Disease Severity. Annals of the American Thoracic Society 2015; 12: 1764-1770
• 165 Anwar GA, McDonnell MJ, Worthy SA. et al. Phenotyping adults with non-cystic fibrosis bronchiectasis: a prospective observational cohort study. Respir Med 2013; 107: 1001-1007
• 166 Aliberti S, Masefield S, Polverino E. et al. Research priorities in bronchiectasis: a consensus statement from the EMBARC Clinical Research Collaboration. Eur Respir J 2016; 48: 632-647
• 167 Raidt J, Brillault J, Brinkmann F. et al. [Management of Primary Ciliary Dyskinesia]. Pneumologie 2020; 74: 750-765
• 168 Murray MP, Pentland JL, Turnbull K. et al. Sputum colour: a useful clinical tool in non-cystic fibrosis bronchiectasis. Eur Respir J 2009; 34: 361-364
• 169 Aliberti S, Ringshausen FC, Dhar R. et al. Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC). Eur Respir J 2024; 63: 2301554
• 170 Patrawala M, Cui Y, Peng LM. et al. Pulmonary Disease Burden in Primary Immune Deficiency Disorders: Data from USIDNET Registry. J Clin Immunol 2020; 40: 340-349
• 171 Gathmann B, Mahlaoui N, Gerard L. et al. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immun 2014; 134: 116-126
• 172 Odnoletkova I, Kindle G, Quinti I. et al. The burden of common variable immunodeficiency disorders: a retrospective analysis of the European Society for Immunodeficiency (ESID) registry data. Orphanet J Rare Dis 2018; 13: 201
• 173 Schutz K, Alecsandru D, Grimbacher B. et al. Imaging of Bronchial Pathology in Antibody Deficiency: Data from the European Chest CT Group. J Clin Immunol 2019; 39: 45-54
• 174 Lucas M, Lee M, Lortan J. et al. Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol 2010; 125: 1354-1360 e1354
• 175 Plebani A, Soresina A, Rondelli R. et al. Clinical, immunological, and molecular analysis in a large cohort of patients with X-linked agammaglobulinemia: An Italian multicenter study. Clin Immunol 2002; 104: 221-230
• 176 Hanitsch L, Baumann U, Boztug K. et al. Treatment and management of primary antibody deficiency: German interdisciplinary evidence-based consensus guideline. Eur J Immunol 2020; 50: 1432-1446
• 177 Gracia J de, Vendrell M, Alvarez A. et al. Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency. Int Immunopharmacol 2004; 4: 745-753
• 178 Tangye SG, Al-Herz W, Bousfiha A. et al. The Ever-Increasing Array of Novel Inborn Errors of Immunity: an Interim Update by the IUIS Committee. J Clin Immunol 2021; 41: 666-679
• 179 Mandal P, Morice AH, Chalmers JD. et al. Symptoms of airway reflux predict exacerbations and quality of life in bronchiectasis. Respir Med 2013; 107: 1008-1013
• 180 Bachmann O, Langer F, Rademacher J. [Pulmonary manifestations of inflammatory bowel disease]. Internist (Berl) 2010; 51 (Suppl. 01) 264-268
• 181 De Soyza A, McDonnell MJ, Goeminne PC. et al. Bronchiectasis Rheumatoid Overlap Syndrome Is an Independent Risk Factor for Mortality in Patients With Bronchiectasis: A Multicenter Cohort Study. Chest 2017; 151: 1247-1254
• 182 Perry E, Eggleton P, De Soyza A. et al. Increased disease activity, severity and autoantibody positivity in rheumatoid arthritis patients with co-existent bronchiectasis. Int J Rheum Dis 2017; 20: 2003-2011
• 183 Quirke AM, Perry E, Cartwright A. et al. Bronchiectasis is a Model for Chronic Bacterial Infection Inducing Autoimmunity in Rheumatoid Arthritis. Arthritis Rheumatol 2015; 67: 2335-2342
• 184 Perry E, Stenton C, Kelly C. et al. RA autoantibodies as predictors of rheumatoid arthritis in non-cystic fibrosis bronchiectasis patients. Eur Respir J 2014; 44: 1082-1085
• 185 Ewen R, Pink I, Sutharsan S. et al. Ciliary Dyskinesia in Adult Bronchiectasis: Data from the German Bronchiectasis Registry PROGNOSIS. Chest 2024;
• 186 McDonnell MJ, Aliberti S, Goeminne PC. et al. Comorbidities and the risk of mortality in patients with bronchiectasis: an international multicentre cohort study. Lancet Respir Med 2016; 4: 969-979
• 187 Rosa Carrillo D de la, Athanazio R, Giron Moreno RM. et al. The annual prognostic ability of FACED and E-FACED scores to predict mortality in patients with bronchiectasis. ERJ Open Res 2018; 4: 00139-2017
• 188 Martinez-Garcia MA, Athanazio R, Gramblicka G. et al. Prognostic Value of Frequent Exacerbations in Bronchiectasis: The Relationship With Disease Severity. Arch Bronconeumol (Engl Ed) 2019; 55: 81-87
• 189 Bundesärztekammer (BÄK), Kassenärztliche Bundesvereinigung (KBV), (AWMF). AdWMF. Nationale VersorgungsLeitlinie Asthma – Langfassung, 4. Auflage. Version 1. www.asthma.versorgungsleitlinien.de Stand: 07.06.2022
• 190 Bundesärztekammer (BÄK), Kassenärztliche Bundesvereinigung (KBV), (AWMF). AdWMF. Nationale VersorgungsLeitlinie COPD – Teilpublikation der Langfassung, 2. Auflage. Version 1. 2021
• 191 Ringshausen FC, Chalmers JD, Pletz MW. Inhaled antibiotics in chronic airway infections. Aliberti S, Chalmers JD, Pletz MW. eds. In: ERS Monograph: Anti-infectives and the Lung. Sheffield: European Respiratory Society; 2017
• 192 Wear S. Informed Consent. Patient autonomy and physician beneficence with clinical medicine. Dordrecht: Kluwer Academic Publishers; 1992
• 193 Leitlinienprogramm Onkologie (Deutsche Krebsgesellschaft; Deutsche Krebshilfe; AWMF). Palliativmedizin für Patienten mit einer nicht-heilbaren Krebserkrankung. https://www.leitlinienprogramm-onkologie.de/leitlinien/palliativmedizin/ (abgerufen am: 20.05.2022);
• 194 Alexander LEC, Shin S, Hwang JH. Inflammatory Diseases of the Lung Induced by Conventional Cigarette Smoke A Review. Chest 2015; 148: 1307-1322
• 195 Mall MA, Criner GJ, Miravitlles M. et al. Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond. Eur Respir J 2023; 61: 2201307
• 196 Zhou ZJ, Chen P, Peng H. Are healthy smokers really healthy?. Tob Induc Dis 2016; 14: 35
• 197 Amin K, Ekberg-Jansson A, Lofdahl CG. et al. Relationship between inflammatory cells and structural changes in the lungs of asymptomatic and never smokers: a biopsy study. Thorax 2003; 58: 135-142
• 198 Yang B, Han K, Kim B. et al. Association between Smoking Status and Incident Non-Cystic Fibrosis Bronchiectasis in Young Adults: A Nationwide Population-Based Study. J Pers Med 2022; 12: 691
• 199 Sin S, Yun SY, Kim JM. et al. Mortality risk and causes of death in patients with non-cystic fibrosis bronchiectasis. Resp Res 2019; 20: 271
• 200 Choi H, Yang B, Kim YJ. et al. Increased mortality in patients with non cystic fibrosis bronchiectasis with respiratory comorbidities. Sci Rep-Uk 2021; 11: 7126
• 201 Navaratnam V, Root AA, Douglas I. et al. Cardiovascular Outcomes after a Respiratory Tract Infection among Adults with Non-Cystic Fibrosis Bronchiectasis: A General Population-based Study. Annals of the American Thoracic Society 2018; 15: 315-321
• 202 Navaratnam V, Millett ER, Hurst JR. et al. Bronchiectasis and the risk of cardiovascular disease: a population-based study. Thorax 2017; 72: 161-166
• 203 Batra A, Kiefer F, Andreas S. et al. S3 Guideline “Smoking and Tobacco Dependence: Screening, Diagnosis, and Treatment” – Short Version. Eur Addict Res 2022; 28: 382-400
• 204 Bundesärztekammer (BÄK), Kassenärztliche Bundesvereinigung (KBV), Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF). Nationale VersorgungsLeitlinie COPD. 2. Auflage, Version 1. 2021
• 205 Riesenhuber M, Aigner K, Neuberger M. et al. Leitfaden zur Tabak- und Nikotinentwöhnung. 1. Auflage. Österreichische Gesellschaft für Pneumologie (ÖGP); 2023
• 206 Brunekreef B, Holgate ST. Air pollution and health. Lancet 2002; 360: 1233-1242
• 207 Rice MB, Ljungman PL, Wilker EH. et al. Short-Term Exposure to Air Pollution and Lung Function in the Framingham Heart Study. Am J Resp Crit Care 2013; 188: 1351-1357
• 208 Lepeule J, Litonjua AA, Coull B. et al. Long-Term Effects of Traffic Particles on Lung Function Decline in the Elderly. Am J Resp Crit Care 2014; 190: 542-548
• 209 Garcia-Olive I, Stojanovic Z, Radua J. et al. Effect of Air Pollution on Exacerbations of Bronchiectasis in Badalona, Spain, 2008–2016. Respiration 2018; 96: 111-116
• 210 Goeminne PC, Bijnens E, Nemery B. et al. Impact of traffic related air pollution indicators on non-cystic fibrosis bronchiectasis mortality: a cohort analysis. Resp Res 2014; 15: 108
• 211 Boucher RC. Muco-Obstructive Lung Diseases. N Engl J Med 2019; 380: 1941-1953
• 212 Lee AL, Burge AT, Holland AE. Airway clearance techniques for bronchiectasis. Cochrane Database Syst Rev 2015; 2015: CD008351
• 213 Bradley J, Moran F, Greenstone M. Physical training for bronchiectasis. Cochrane Database Syst Rev 2002; CD002166
• 214 Herrero-Cortina B, Lee AL, Oliveira A. et al. European Respiratory Society statement on airway clearance techniques in adults with bronchiectasis. Eur Respir J 2023; 62: 2202053
• 215 Lee AL, Burge AT, Holland AE. Airway clearance techniques for bronchiectasis. Cochrane Db Syst Rev 2015;
• 216 Patterson JE, Bradley JM, Hewitt O. et al. Airway clearance in bronchiectasis: A randomized crossover trial of active cycle of breathing techniques versus Acapella (R). Respiration 2005; 72: 239-242
• 217 Figueiredo PH, Zin WA, Guimaraes FS. Flutter valve improves respiratory mechanics and sputum production in patients with bronchiectasis. Physiother Res Int 2012; 17: 12-20
• 218 Guimaraes FS, Moco VJR, Menezes SLS. et al. Effects of ELTGOL and Flutter VRP1 (R) on the dynamic and static pulmonary volumes and on the secretion clearance of patients with bronchiectasis. Braz J Phys Ther 2012; 16: 108-113
• 219 Herrero-Cortina B, Vilaro J, Marti D. et al. Short-term effects of three slow expiratory airway clearance techniques in patients with bronchiectasis: a randomised crossover trial. Physiotherapy 2016; 102: 357-364
• 220 Anuradha K, Gunathilaka PKG, Wickramasinghe VP. Effectiveness of hypertonic saline nebulization in airway clearance in children with non-cystic fibrosis bronchiectasis: A randomized control trial. Pediatr Pulmonol 2021; 56: 509-515
• 221 Herrero-Cortina B, Alcaraz V, Vilaro J. et al. Impact of Hypertonic Saline Solutions on Sputum Expectoration and Their Safety Profile in Patients with Bronchiectasis: A Randomized Crossover Trial. J Aerosol Med Pulm Drug Deliv 2018; 31: 281-289
• 222 Spinou A, Herrero-Cortina B, Aliberti S. et al. Airway clearance management in people with bronchiectasis: data from the European Bronchiectasis Registry (EMBARC). Eur Respir J 2024; 63: 2301689
• 223 McCullough AR, Tunney MM, Quittner AL. et al. Treatment adherence and health outcomes in patients with bronchiectasis. BMC Pulm Med 2014; 14: 107
• 224 McIlwaine M, Bradley J, Elborn JS. et al. Personalising airway clearance in chronic lung disease. European Respiratory Review 2017; 26: 160086
• 225 Hester KLM, Newton J, Rapley T. et al. Patient information, education and self-management in bronchiectasis: facilitating improvements to optimise health outcomes. BMC Pulm Med 2018; 18: 80
• 226 Haworth CS, Foweraker JE, Wilkinson P. et al. Inhaled colistin in patients with bronchiectasis and chronic Pseudomonas aeruginosa infection. Am J Respir Crit Care Med 2014; 189: 975-982
• 227 OʼDonnell AE. Airway Clearance and Mucoactive Therapies in Bronchiectasis. Clin Chest Med 2022; 43: 157-163
• 228 Davison J, Robinson-Barella A, Davies G. et al. Patient attitudes to nebulised antibiotics in the treatment of bronchiectasis: a mixed-methods study. ERJ Open Res 2023; 9: 00735-2022
• 229 Spruit MA, Singh SJ, Garvey C. et al. An Official American Thoracic Society/European Respiratory Society Statement: Key Concepts and Advances in Pulmonary Rehabilitation. Am J Resp Crit Care 2013; 188: E13-E64
• 230 Bull FC, Al-Ansari SS, Biddle S. et al. World Health Organization 2020 guidelines on physical activity and sedentary behaviour. Br J Sports Med 2020; 54: 1451-1462
• 231 Lee AL, Hill CJ, Cecins N. et al. The short and long term effects of exercise training in non-cystic fibrosis bronchiectasis – a randomised controlled trial. Respir Res 2014; 15: 44
• 232 Alcaraz-Serrano V, Gimeno-Santos E, Scioscia G. et al. Association between physical activity and risk of hospitalisation in bronchiectasis. Eur Respir J 2020; 55: 1902138
• 233 Schultz K, Koczulla AR. [Pulmonary Rehabilitation in Patients with Chronic Obstructive Airway Diseases]. Pneumologie 2021; 75: 457-473
• 234 McShane PJ, Naureckas ET, Tino G. et al. Non-Cystic Fibrosis Bronchiectasis. Am J Resp Crit Care 2013; 188: 647-656
• 235 Chang AB, Bell SC, Torzillo PJ. et al. Thoracic Society of Australia and New Zealand guidelines Chronic suppurative lung disease and bronchiectasis in children and adults in Australia and New Zealand. Med J Australia 2015; 202: 21-U46
• 236 Watson KE, Lee AL, Dwyer TJ. et al. Applying the treatable traits approach in bronchiectasis-A scoping review of traits, measurements and treatments implemented by allied health professionals and nurses. Respir Med 2023; 222: 107503
• 237 Zhang P, Jiang GN, Ding JA. et al. Surgical Treatment of Bronchiectasis: A Retrospective Analysis of 790 Patients. Ann Thorac Surg 2010; 90: 246-251
• 238 Borrie J, Lichter I. Surgical treatment of bronchiectasis: ten-year survey. Br Med J 1965; 2: 908-912
• 239 Dogan R, Alp M, Kaya S. et al. Surgical-Treatment of Bronchiectasis – a Collective Review of 487 Cases. Thorac Cardiov Surg 1989; 37: 183-186
• 240 Ashour M, AlKattan KM, Jain SK. et al. Surgery for unilateral bronchiectasis: Results and prognostic factors. Tubercle Lung Dis 1996; 77: 168-172
• 241 Smit HJM, Schreurs AJM, VandenBosch JMM. et al. Is resection of bronchiectasis beneficial in patients with primary ciliary dyskinesia?. Chest 1996; 109: 1541-1544
• 242 Agasthian T, Deschamps C, Trastek VF. et al. Surgical management of bronchiectasis. Ann Thorac Surg 1996; 62: 976-978
• 243 Halezeroglu S, Keles M, Uysal A. et al. Factors affecting postoperative morbidity and mortality in destroyed lung. Ann Thorac Surg 1997; 64: 1635-1638
• 244 Balkanli K, Genc O, Dakak M. et al. Surgical management of bronchiectasis: analysis and short-term results in 238 patients. Eur J Cardio-Thorac 2003; 24: 699-702
• 245 Eren S, Esme H, Avci A. Risk factors affecting outcome and morbidity in the surgical management of bronchiectasis. J Thorac Cardiov Sur 2007; 134: 392-398
• 246 Giovannetti R, Alifano M, Stefani A. et al. Surgical treatment of bronchiectasis: early and long-term results. Interact Cardiovasc Thorac Surg 2008; 7: 609-612
• 247 Bagheri R, Haghi SZ, Masoum SHF. et al. Surgical Management of Bronchiectasis: Analysis of 277 Patients. Thorac Cardiov Surg 2010; 58: 291-294
• 248 Gorur R, Turut H, Yiyit N. et al. The Influence of Specific Factors on Postoperative Morbidity in Young Adults with Bronchiectasis. Heart Lung Circ 2011; 20: 468-472
• 249 Caylak H, Genc O, Kavakli K. et al. Surgical Management of Bronchiectasis: A Collective Review of 339 Patients with Long-term Follow-up. Thorac Cardiov Surg 2011; 59: 479-483
• 250 Cobanoglu U, Yalcinkaya I, Er M. et al. Surgery for bronchiectasis: The effect of morphological types to prognosis. Ann Thorac Med 2011; 6: 25-32
• 251 Sehitogullari A, Bilici S, Sayir F. et al. A long-term study assessing the factors influencing survival and morbidity in the surgical management of bronchiectasis. J Cardiothorac Surg 2011; 6: 161
• 252 Zhang P, Zhang FJ, Jiang SM. et al. Video-Assisted Thoracic Surgery for Bronchiectasis. Ann Thorac Surg 2011; 91: 239-243
• 253 Hiramatsu M, Shiraishi Y, Nakajima Y. et al. Risk Factors That Affect the Surgical Outcome in the Management of Focal Bronchiectasis in a Developed Country. Ann Thorac Surg 2012; 93: 245-250
• 254 Zhou ZL, Zhao H, Li Y. et al. Completely thoracoscopic lobectomy for the surgical management of bronchiectasis. Chinese Med J-Peking 2013; 126: 875-878
• 255 Balci AE, Balci TA, Ozyurtan MO. Current Surgical Therapy for Bronchiectasis: Surgical Results and Predictive Factors in 86 Patients. Ann Thorac Surg 2014; 97: 211-217
• 256 Rademacher J, Schweigert M. [Therapy of bronchiectasis]. Zentralbl Chir 2015; 140: 113-126
• 257 Fan LC, Liang S, Lu HW. et al. Efficiency and safety of surgical intervention to patients with Non-Cystic Fibrosis bronchiectasis: a meta-analysis. Sci Rep-Uk 2015; 5: 17382
• 258 Dai J, Zhu XS, Bian DL. et al. Surgery for predominant lesion in nonlocalized bronchiectasis. J Thorac Cardiov Sur 2017; 153: 979-985
• 259 Baysungur V, Dogruyol T, Ocakcioglu I. et al. The Feasibility of Thoracoscopic Resection in Bronchiectasis. Surg Laparo Endo Per 2017; 27: 194-196
• 260 Hiramatsu M, Shiraishi Y. Surgical management of non-cystic fibrosis bronchiectasis. J Thorac Dis 2018; 10: S3436-S3445
• 261 Gulhan SSE, Acar LN, Guven ES. et al. Surgical treatment of bronchiectasis: Our 23 years of experience. Turk Gogus Kalp Dama 2020; 28: 629-637
• 262 Selman A, Merhej H, Nakagiri T. et al. Surgical treatment of non-cystic fibrosis bronchiectasis in Central Europe. J Thorac Dis 2021; 13: 5843-5850
• 263 Westphal FL, Lima LC de, Netto JCL. et al. Carcinoid tumor and pulmonary sequestration. Jornal Brasileiro De Pneumologia 2012; 38: 133-137
• 264 Li M, Zhang GL, Peng AM. et al. Bronchial Fibroepithelial Polyp: A Case Report and Review of the Literature. Internal Med 2013; 52: 373-376
• 265 Morgenthau AS, Teirstein AS. Sarcoidosis of the upper and lower airways. Expert Rev Resp Med 2011; 5: 823-833
• 266 Javidan-Nejad C, Bhalla S. Bronchiectasis. Radiol Clin North Am 2009; 47: 289-306
• 267 Einarsson JT, Einarsson JG, Isaksson H. et al. Middle lobe syndrome: a nationwide study on clinicopathological features and surgical treatment. Clin Respir J 2009; 3: 77-81
• 268 Long QH, Zha YF, Yang ZG. Evaluation of pulmonary sequestration with multidetector computed tomography angiography in a select cohort of patients: A retrospective study. Clinics 2016; 71: 392-398
• 269 Cordovilla R, Bollo de Miguel E, Nunez Ares A. et al. Diagnosis and Treatment of Hemoptysis. Arch Bronconeumol 2016; 52: 368-377
• 270 Fruchter O, Schneer S, Rusanov V. et al. Bronchial artery embolization for massive hemoptysis: long-term follow-up. Asian Cardiovasc Thorac Ann 2015; 23: 55-60
• 271 Farjah F, Symons RG, Krishnadasan B. et al. Management of pleural space infections: A population-based analysis. J Thorac Cardiov Sur 2007; 133: 346-U310
• 272 Fujimoto T, Hillejan L, Stamatis G. Current strategy for surgical management of bronchiectasis. Ann Thorac Surg 2001; 72: 1711-1715
• 273 Milman S, Ng T. Experience and patient selection are the keys to successful surgical treatment for nonlocalized bronchiectasis. J Thorac Cardiov Sur 2017; 153: 986-986
• 274 Schneiter D, Meyer N, Lardinois D. et al. Surgery for non-localized bronchiectasis. Brit J Surg 2005; 92: 836-839
• 275 Brunelli A, Cicconi S, Decaluwe H. et al. Parsimonious Eurolung risk models to predict cardiopulmonary morbidity and mortality following anatomic lung resections: an updated analysis from the European Society of Thoracic Surgeons database. Eur J Cardio-Thorac 2020; 57: 455-461
• 276 Tilburg PMB van, Stam H, Hoogsteden HC. et al. Pre-operative pulmonary evaluation of lung cancer patients: a review of the literature. European Respiratory Journal 2009; 33: 1206-1215
• 277 Mohamadiyeh MKH, Ashour M, Eldesouki M. et al. Contribution of Ventilation and Perfusion Lung Imaging to the Management of Patients with Bronchiectasis. Clin Nucl Med 1994; 19: 292-297
• 278 Yoo ID, Im JJ, Chung YA. et al. Prediction of postoperative lung function in lung cancer patients using perfusion scintigraphy. Acta Radiol 2019; 60: 488-495
• 279 Warburton CJ, Corless JA. Surgery vs non-surgical treatment for bronchiectasis. Cochrane Database Syst Rev 2000; CD002180
• 280 Demmy TL, Nwogu C. Is video-assisted thoracic surgery lobectomy better? Quality of life considerations. Ann Thorac Surg 2008; 85: S719-S728
• 281 Weber A, Stammberger U, Inci I. et al. Thoracoscopic lobectomy for benign disease – a single centre study on 64 cases. Eur J Cardio-Thorac 2001; 20: 443-447
• 282 Usuda K, Maeda S, Motomo N. et al. Pulmonary Function After Lobectomy: Video-Assisted Thoracoscopic Surgery Versus Muscle-Sparing Mini-thoracotomy. Indian J Surg 2017; 79: 504-509
• 283 McKenna Jr RJ. Complications and learning curves for video-assisted thoracic surgery lobectomy. Thorac Surg Clin 2008; 18: 275-280
• 284 Yan TD, Cao C, DʼAmico TA. et al. Video-assisted thoracoscopic surgery lobectomy at 20 years: a consensus statement. Eur J Cardio-Thorac 2014; 45: 633-639
• 285 Gursoy S, Ozturk AA, Ucvet A. et al. Surgical management of bronchiectasis: The indications and outcomes. Surg Today 2010; 40: 26-30
• 286 Windisch W, Dreher M, Geiseler J. et al. [Guidelines for Non-Invasive and Invasive Home Mechanical Ventilation for Treatment of Chronic Respiratory Failure – Update 2017]. Pneumologie 2017; 71: 722-795
• 287 Holland AE, Denehy L, Ntoumenopoulos G. et al. Non-invasive ventilation assists chest physiotherapy in adults with acute exacerbations of cystic fibrosis. Thorax 2003; 58: 880-884
• 288 Young AC, Wilson JW, Kotsimbos TC. et al. Randomised placebo controlled trial of non-invasive ventilation for hypercapnia in cystic fibrosis. Thorax 2008; 63: 72-77
• 289 Fauroux B, Le Roux E, Ravilly S. et al. Long-term noninvasive ventilation in patients with cystic fibrosis. Respiration 2008; 76: 168-174
• 290 Moran F, Bradley JM, Piper AJ. Non-invasive ventilation for cystic fibrosis. Cochrane Database Syst Rev 2017; 2: CD002769
• 291 Chambers DC, Perch M, Zuckermann A. et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-eighth adult lung transplantation report – 2021; Focus on recipient characteristics. J Heart Lung Transplant 2021; 40: 1060-1072
• 292 Leard LE, Holm AM, Valapour M. et al. Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. J Heart Lung Transplant 2021; 40: 1349-1379
• 293 Beirne PA, Banner NR, Khaghani A. et al. Lung transplantation for non-cystic fibrosis bronchiectasis: analysis of a 13-year experience. J Heart Lung Transplant 2005; 24: 1530-1535
• 294 Nathan JA, Sharples LD, Exley AR. et al. The outcomes of lung transplantation in patients with bronchiectasis and antibody deficiency. J Heart Lung Transplant 2005; 24: 1517-1521
• 295 Titman A, Rogers CA, Bonser RS. et al. Disease-specific survival benefit of lung transplantation in adults: a national cohort study. Am J Transplant 2009; 9: 1640-1649
• 296 Hayes D, Kopp BT, Tobias JD. et al. Survival in Patients with Advanced Non-cystic Fibrosis Bronchiectasis Versus Cystic Fibrosis on the Waitlist for Lung Transplantation. Lung 2015; 193: 933-938
• 297 Hayes Jr D, Reynolds SD, Tumin D. Outcomes of lung transplantation for primary ciliary dyskinesia and Kartagener syndrome. J Heart Lung Transplant 2016; 35: 1377-1378
• 298 Rademacher J, Ringshausen FC, Suhling H. et al. Lung transplantation for non-cystic fibrosis bronchiectasis. Respir Med 2016; 115: 60-65
• 299 Birch J, Sunny SS, Hester KLM. et al. Outcomes of lung transplantation in adults with bronchiectasis. BMC Pulm Med 2018; 18: 82
• 300 Hirama T, Tomiyama F, Notsuda H. et al. Outcome and prognostic factors after lung transplantation for bronchiectasis other than cystic fibrosis. Bmc Pulmonary Medicine 2021; 21: 261
• 301 Kennedy JL, Walker A, Ellender CM. et al. Outcomes of non-cystic fibrosis-related bronchiectasis post-lung transplantation. Intern Med J 2022; 52: 995-1001
• 302 Vayvada M, Gordebil A, Saribas E. et al. Lung transplantation for non-cystic fibrosis bronchiectasis in Turkey: An initial institutional experience. Asian J Surg 2022; 45: 162-166
• 303 Batram M, Witte J, Schwarz M. et al. Burden of Herpes Zoster in Adult Patients with Underlying Conditions: Analysis of German Claims Data, 2007–2018. Dermatology Ther 2021; 11: 1009-1026
• 304 Bahrs C, Kesselmeier M, Kolditz M. et al. A longitudinal analysis of pneumococcal vaccine serotypes in pneumonia patients in Germany. European Respiratory Journal 2022; 59: 2102432
• 305 Kessel DA van, Velzen-Blad H van, Bosch JMM van den. et al. Impaired pneumococcal antibody response in bronchiectasis of unknown aetiology. European Respiratory Journal 2005; 25: 482-489
• 306 Aveyard P, Gao M, Lindson N. et al. Association between pre-existing respiratory disease and its treatment, and severe COVID-19: a population cohort study. Lancet Resp Med 2021; 9: 909-923
• 307 Shteinberg M, Sibila O, Stein N. et al. Risk of SARS-CoV-2 infection and disease severity among people with bronchiectasis: analysis of three population registries. European Respiratory Journal 2023; 62: PA5098
• 308 Lommatzsch M, Rabe KF, Taube C. et al. Risk Assessment for Patients with Chronic Respiratory Conditions in the Context of the SARS-CoV-2 Pandemic Statement of the German Respiratory Society with the Support of the German Association of Chest Physicians. Respiration 2022; 101: 307-320
• 309 Shi T, Vennard S, Jasiewicz F. et al. Disease Burden Estimates of Respiratory Syncytial Virus related Acute Respiratory Infections in Adults With Comorbidity: A Systematic Review and Meta-Analysis. J Infect Dis 2022; 226: S17-S21
• 310 Savic M, Penders Y, Shi T. et al. Respiratory syncytial virus disease burden in adults aged 60 years and older in high-income countries: A systematic literature review and meta-analysis. Influenza Other Respir Viruses 2023; 17: e13031
• 311 Shi T, Denouel A, Tietjen AK. et al. Global Disease Burden Estimates of Respiratory Syncytial Virus-Associated Acute Respiratory Infection in Older Adults in 2015: A Systematic Review and Meta-Analysis. J Infect Dis 2020; 222: S577-S583
• 312 Nazareno AL, Muscatello DJ, Turner RM. et al. Modelled estimates of hospitalisations attributable to respiratory syncytial virus and influenza in Australia, 2009–2017. Influenza Other Respir Viruses 2022; 16: 1082-1090
• 313 Deutsche Gesellschaft für Pneumologie und Beatmungsmedizin (DGP) e. V.. Positionspapier zur RSV-Schutzimpfung bei besonders gefährdeten Patientinnen und Patienten. https://pneumologie.de/storage/app/media/uploaded-files/2023_RSV-Impfung_DGP.pdf Letzter Zugriff am 01.02.2024
• 314 Ringshausen FC, Shapiro AJ, Nielsen KG. et al. Safety and efficacy of the epithelial sodium channel blocker idrevloride in people with primary ciliary dyskinesia (CLEAN-PCD): a multinational, phase 2, randomised, double-blind, placebo-controlled crossover trial. Lancet Respir Med 2024; 12: 21-33
• 315 Ramsey KA, Chen ACH, Radicioni G. et al. Airway Mucus Hyperconcentration in Non-Cystic Fibrosis Bronchiectasis. Am J Respir Crit Care Med 2020; 201: 661-670
• 316 Nair GB, Ilowite JS. Pharmacologic agents for mucus clearance in bronchiectasis. Clin Chest Med 2012; 33: 363-370
• 317 Hart A, Sugumar K, Milan SJ. et al. Inhaled hyperosmolar agents for bronchiectasis. Cochrane Database Syst Rev 2014; 2014: CD002996
• 318 Xie B, Liu P, Wu Q. et al. The efficacy of inhaled hypertonic saline for bronchiectasis: a meta-analysis of randomized controlled studies. Am J Emerg Med 2020; 38: 2713-2717
• 319 Kellett F, Redfern J, Niven RM. Evaluation of nebulised hypertonic saline (7%) as an adjunct to physiotherapy in patients with stable bronchiectasis. Respir Med 2005; 99: 27-31
• 320 Kellett F, Robert NM. Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis. Respir Med 2011; 105: 1831-1835
• 321 Nicolson CH, Stirling RG, Borg BM. et al. The long term effect of inhaled hypertonic saline 6% in non-cystic fibrosis bronchiectasis. Respir Med 2012; 106: 661-667
• 322 Shteinberg M, Spinou A, Goeminne P. et al. Prescribing preferences and availability of nebulisers and inhalers for inhaled medications in bronchiectasis: results of a specialist survey. ERJ Open Res 2024; 10: 00724-2023
• 323 Gemeinsamer Bundesausschuss (G-BA). Anlage V zum Abschnitt J der Arzneimittel-Richtlinie: Übersicht der verordnungsfähigen Medizinprodukte. https://www.g-ba.de/downloads/83-691-868/AM-RL-V_2024-01-23.pdf Letzter Zugriff am 02.02.2024
• 324 Tarrant BJ, Le Maitre C, Romero L. et al. Mucoactive agents for chronic, non-cystic fibrosis lung disease: A systematic review and meta-analysis. Respirology 2017; 22: 1084-1092
• 325 Bilton D, Daviskas E, Anderson SD. et al. Phase 3 randomized study of the efficacy and safety of inhaled dry powder mannitol for the symptomatic treatment of non-cystic fibrosis bronchiectasis. Chest 2013; 144: 215-225
• 326 Bilton D, Tino G, Barker AF. et al. Inhaled mannitol for non-cystic fibrosis bronchiectasis: a randomised, controlled trial. Thorax 2014; 69: 1073-1079
• 327 Gao YH, AboLeyah H, Finch S. et al. Relationship between Symptoms, Exacerbations, and Treatment Response in Bronchiectasis. Am J Respir Crit Care Med 2020; 201: 1499-1507
• 328 OʼDonnell AE, Barker AF, Ilowite JS. et al. Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group. Chest 1998; 113: 1329-1334
• 329 Wills PJ, Wodehouse T, Corkery K. et al. Short-term recombinant human DNase in bronchiectasis. Effect on clinical state and in vitro sputum transportability. Am J Respir Crit Care Med 1996; 154: 413-417
• 330 Wilkinson M, Sugumar K, Milan SJ. et al. Mucolytics for bronchiectasis. Cochrane Database Syst Rev 2014; 2014: CD001289
• 331 Olivieri D, Ciaccia A, Marangio E. et al. Role of bromhexine in exacerbations of bronchiectasis. Double-blind randomized multicenter study versus placebo. Respiration 1991; 58: 117-121
• 332 Crisafulli E, Coletti O, Costi S. et al. Effectiveness of erdosteine in elderly patients with bronchiectasis and hypersecretion: a 15-day, prospective, parallel, open-label, pilot study. Clin Ther 2007; 29: 2001-2009
• 333 Qi Q, Ailiyaer Y, Liu R. et al. Effect of N-acetylcysteine on exacerbations of bronchiectasis (BENE): a randomized controlled trial. Respir Res 2019; 20: 73
• 334 Hill AT, Welham S, Reid K. et al. British Thoracic Society national bronchiectasis audit 2010 and 2011. Thorax 2012; 67: 928-930
• 335 Franco F, Sheikh A, Greenstone M. Short acting beta-2 agonists for bronchiectasis. Cochrane Database Syst Rev 2003; CD003572
• 336 Goyal V, Chang AB. Combination inhaled corticosteroids and long-acting beta2-agonists for children and adults with bronchiectasis. Cochrane Database Syst Rev 2014; 2014: CD010327
• 337 Lasserson T, Holt K, Evans D. et al. Anticholinergic therapy for bronchiectasis. Cochrane Database Syst Rev 2001; 2001: CD002163
• 338 Jayaram L, Vandal AC, Chang CL. et al. Tiotropium treatment for bronchiectasis: a randomised, placebo-controlled, crossover trial. Eur Respir J 2022; 59: 2102184
• 339 Martinez-Garcia MA, Soler-Cataluna JJ, Catalan-Serra P. et al. Clinical efficacy and safety of budesonide-formoterol in non-cystic fibrosis bronchiectasis. Chest 2012; 141: 461-468
• 340 Veer T van der, Koning Gans JM de, Braunstahl GJ. et al. The effect of beclomethasone-formoterol versus placebo on chronic cough in patients with non-CF bronchiectasis: the FORZA randomised controlled trial. Eur Respir J 2023; 61: 2300186
• 341 Agusti A, Celli BR, Criner GJ. et al. Global Initiative for Chronic Obstructive Lung Disease 2023 Report: GOLD Executive Summary. Eur Respir J 2023; 61: 2300239
• 342 Global Initiative for Asthma (GINA). Global Strategy for Asthma Management and Prevention. 2023 https://ginasthma.org/wp-content/uploads/2023/07/GINA-2023-Full-report-23_07_06-WMS.pdf Letzter Zugriff am 04.02.2023
• 343 Lommatzsch M, Criee CP, Jong CCM de. et al. [Diagnosis and treatment of asthma: a guideline for respiratory specialists 2023 – published by the German Respiratory Society (DGP) e. V.]. Pneumologie 2023; 77: 461-543
• 344 Hassan JA, Saadiah S, Roslan H. et al. Bronchodilator response to inhaled beta-2 agonist and anticholinergic drugs in patients with bronchiectasis. Respirology 1999; 4: 423-426
• 345 Tsang KW, Tan KC, Ho PL. et al. Inhaled fluticasone in bronchiectasis: a 12 month study. Thorax 2005; 60: 239-243
• 346 Kapur N, Bell S, Kolbe J. et al. Inhaled steroids for bronchiectasis. Cochrane Database Syst Rev 2009; CD000996
• 347 Hernando R, Drobnic ME, Cruz MJ. et al. Budesonide efficacy and safety in patients with bronchiectasis not due to cystic fibrosis. Int J Clin Pharm 2012; 34: 644-650
• 348 Lasserson T, Holt K, Greenstone M. Oral steroids for bronchiectasis (stable and acute exacerbations). Cochrane Database Syst Rev 2001; 2001: CD002162
• 349 Steele K, Greenstone M, Lasserson JA. Oral methyl-xanthines for bronchiectasis. Cochrane Database Syst Rev 2001; 2000: CD002734
• 350 Juthong S, Panyarath P. Efficacy of Roflumilast in Bronchiectasis Patients with Frequent Exacerbations: A Double-Blinded, Randomized, Placebo-Controlled Pilot Clinical Trial. Tuberc Respir Dis (Seoul) 2022; 85: 67-73
• 351 Kapur N, Chang AB. Oral non steroid anti-inflammatories for children and adults with bronchiectasis. Cochrane Database Syst Rev 2007; CD006427
• 352 Pizzutto SJ, Upham JW, Yerkovich ST. et al. Inhaled non-steroid anti-inflammatories for children and adults with bronchiectasis. Cochrane Database Syst Rev 2016; 2016: CD007525
• 353 Tamaoki J, Chiyotani A, Kobayashi K. et al. Effect of indomethacin on bronchorrhea in patients with chronic bronchitis, diffuse panbronchiolitis, or bronchiectasis. Am Rev Respir Dis 1992; 145: 548-552
• 354 Corless JA, Warburton CJ. Leukotriene receptor antagonists for non-cystic fibrosis bronchiectasis. Cochrane Database Syst Rev 2000; 2000: CD002174
• 355 Mandal P, Chalmers JD, Graham C. et al. Atorvastatin as a stable treatment in bronchiectasis: a randomised controlled trial. Lancet Respir Med 2014; 2: 455-463
• 356 Bedi P, Chalmers JD, Graham C. et al. A Randomized Controlled Trial of Atorvastatin in Patients With Bronchiectasis Infected With Pseudomonas Aeruginosa: A Proof of Concept Study. Chest 2017; 152: 368-378
• 357 De Soyza A, Pavord I, Elborn JS. et al. A randomised, placebo-controlled study of the CXCR2 antagonist AZD5069 in bronchiectasis. Eur Respir J 2015; 46: 1021-1032
• 358 Stockley R, De Soyza A, Gunawardena K. et al. Phase II study of a neutrophil elastase inhibitor (AZD9668) in patients with bronchiectasis. Respir Med 2013; 107: 524-533
• 359 Watz H, Nagelschmitz J, Kirsten A. et al. Safety and efficacy of the human neutrophil elastase inhibitor BAY 85-8501 for the treatment of non-cystic fibrosis bronchiectasis: A randomized controlled trial. Pulm Pharmacol Ther 2019; 56: 86-93
• 360 Chalmers JD, Haworth CS, Metersky ML. et al. Phase 2 Trial of the DPP-1 Inhibitor Brensocatib in Bronchiectasis. N Engl J Med 2020; 383: 2127-2137
• 361 Chalmers JD, Gupta A, Chotirmall SH. et al. A Phase 2 randomised study to establish efficacy, safety and dosing of a novel oral cathepsin C inhibitor, BI 1291583, in adults with bronchiectasis: Airleaf. ERJ Open Res 2023; 9: 00633-2022
• 362 Elborn JS, Johnston B, Allen F. et al. Inhaled steroids in patients with bronchiectasis. Respir Med 1992; 86: 121-124
• 363 Martinez-Garcia MA, Perpina-Tordera M, Roman-Sanchez P. et al. Inhaled steroids improve quality of life in patients with steady-state bronchiectasis. Respir Med 2006; 100: 1623-1632
• 364 Tsang KW, Ho PL, Lam WK. et al. Inhaled fluticasone reduces sputum inflammatory indices in severe bronchiectasis. Am J Respir Crit Care Med 1998; 158: 723-727
• 365 Tsang KW, Tan KC, Ho PL. et al. Exhaled nitric oxide in bronchiectasis: the effects of inhaled corticosteroid therapy. Int J Tuberc Lung Dis 2004; 8: 1301-1307
• 366 Kapur N, Petsky HL, Bell S. et al. Inhaled corticosteroids for bronchiectasis. Cochrane Database Syst Rev 2018; 5: CD000996
• 367 Ritchie AI, Singayagam A, Mitchell S. et al. The Effect of Inhaled Corticosteroids on Pneumonia Risk in Patients With COPD-Bronchiectasis Overlap: A UK Population-Based Case-Control Study. Chest 2023; 164: 875-884
• 368 Holme J, Tomlinson JW, Stockley RA. et al. Adrenal suppression in bronchiectasis and the impact of inhaled corticosteroids. Eur Respir J 2008; 32: 1047-1052
• 369 Andrejak C, Nielsen R, Thomsen VO. et al. Chronic respiratory disease, inhaled corticosteroids and risk of non-tuberculous mycobacteriosis. Thorax 2013; 68: 256-262
• 370 Brode SK, Campitelli MA, Kwong JC. et al. The risk of mycobacterial infections associated with inhaled corticosteroid use. Eur Respir J 2017; 50: 1700037
• 371 Chalmers JD, Laska IF, Franssen FME. et al. Withdrawal of inhaled corticosteroids in COPD: a European Respiratory Society guideline. Eur Respir J 2020; 55: 2000351
• 372 Oriano M, Gramegna A, Amati F. et al. T2-High Endotype and Response to Biological Treatments in Patients with Bronchiectasis. Biomedicines 2021; 9: 772
• 373 Martineau AR, Jolliffe DA, Hooper RL. et al. Vitamin D supplementation to prevent acute respiratory tract infections: systematic review and meta-analysis of individual participant data. BMJ 2017; 356: i6583
• 374 Bartley J, Garrett J, Camargo Jr CA. et al. Vitamin D(3) supplementation in adults with bronchiectasis: A pilot study. Chron Respir Dis 2018; 15: 384-392
• 375 Chalmers JD, McHugh BJ, Docherty C. et al. Vitamin-D deficiency is associated with chronic bacterial colonisation and disease severity in bronchiectasis. Thorax 2013; 68: 39-47
• 376 Jolliffe DA, Camargo Jr CA, Sluyter JD. et al. Vitamin D supplementation to prevent acute respiratory infections: a systematic review and meta-analysis of aggregate data from randomised controlled trials. Lancet Diabetes Endocrinol 2021; 9: 276-292
• 377 Jolliffe DA, Greenberg L, Hooper RL. et al. Vitamin D to prevent exacerbations of COPD: systematic review and meta-analysis of individual participant data from randomised controlled trials. Thorax 2019; 74: 337-345
• 378 Gao YH, Zheng HZ, Lu HW. et al. Quality of Life Bronchiectasis Respiratory Symptom Predicts the Risk of Exacerbations in Adults with Bronchiectasis: A Prospective Observational Study. Annals of the American Thoracic Society 2024; 21: 393-401
• 379 Chalmers JD, Boersma W, Lonergan M. et al. Long-term macrolide antibiotics for the treatment of bronchiectasis in adults: an individual participant data meta-analysis. Lancet Respir Med 2019; 7: 845-854
• 380 Laska IF, Crichton ML, Shoemark A. et al. The efficacy and safety of inhaled antibiotics for the treatment of bronchiectasis in adults: a systematic review and meta-analysis. Lancet Respir Med 2019; 7: 855-869
• 381 Cordeiro R, Choi H, Haworth CS. et al. The efficacy and safety of inhaled antibiotics for the treatment of bronchiectasis in adults: Updated systematic review and meta-analysis. Chest 2024;
• 382 Kelly C, Chalmers JD, Crossingham I. et al. Macrolide antibiotics for bronchiectasis. Cochrane Database Syst Rev 2018; 3: CD012406
• 383 Serisier DJ, Martin ML, McGuckin MA. et al. Effect of long-term, low-dose erythromycin on pulmonary exacerbations among patients with non-cystic fibrosis bronchiectasis: the BLESS randomized controlled trial. JAMA: the journal of the American Medical Association 2013; 309: 1260-1267
• 384 Altenburg J, Graaff CS de, Stienstra Y. et al. Effect of azithromycin maintenance treatment on infectious exacerbations among patients with non-cystic fibrosis bronchiectasis: the BAT randomized controlled trial. JAMA: the journal of the American Medical Association 2013; 309: 1251-1259
• 385 Murray MP, Govan JR, Doherty CJ. et al. A randomized controlled trial of nebulized gentamicin in non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med 2011; 183: 491-499
• 386 Aksamit T, De Soyza A, Bandel TJ. et al. RESPIRE 2: a phase III placebo-controlled randomised trial of ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis. Eur Respir J 2018; 51: 1702053
• 387 De Soyza A, Aksamit T, Bandel TJ. et al. RESPIRE 1: a phase III placebo-controlled randomised trial of ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis. Eur Respir J 2018; 51: 1702052
• 388 Haworth CS, Bilton D, Chalmers JD. et al. Inhaled liposomal ciprofloxacin in patients with non-cystic fibrosis bronchiectasis and chronic lung infection with Pseudomonas aeruginosa (ORBIT-3 and ORBIT-4): two phase 3, randomised controlled trials. Lancet Respir Med 2019; 7: 213-226
• 389 Haworth CS, Shteinberg M, Winthrop KL. et al. RCT Abstract – The efficacy and safety of colistimethate sodium delivered via the I-neb in bronchiectasis: the PROMIS-I randomized controlled trial. European Respiratory Journal 2021; 58: RCT4267
• 390 Loebinger MR, Polverino E, Chalmers JD. et al. Efficacy and safety of TOBI Podhaler in Pseudomonas aeruginosa-infected bronchiectasis patients: iBEST study. Eur Respir J 2021; 57: 2001451
• 391 Burgel PR, Southern KW, Addy C. et al. Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues. J Cyst Fibros 2024; 23: P187-202
• 392 Smith S, Rowbotham NJ. Inhaled anti-pseudomonal antibiotics for long-term therapy in cystic fibrosis. Cochrane Database Syst Rev 2022; 11: CD001021
• 393 Griffith DE, Eagle G, Thomson RM. et al. Randomized Phase 3 Trial of Amikacin Liposome Inhalation Suspension (ALIS) for Treatment-Refractory Nontuberculous Mycobacterial (NTM) Lung Disease Caused by Mycobacterium Avium Complex (MAC) in Adult Patients. Am J Resp Crit Care 2018; 197: A5915
• 394 Orriols R, Roig J, Ferrer J. et al. Inhaled antibiotic therapy in non-cystic fibrosis patients with bronchiectasis and chronic bronchial infection by Pseudomonas aeruginosa. Respir Med 1999; 93: 476-480
• 395 Barker AF, Couch L, Fiel SB. et al. Tobramycin solution for inhalation reduces sputum Pseudomonas aeruginosa density in bronchiectasis. Am J Respir Crit Care Med 2000; 162: 481-485
• 396 Drobnic ME, Sune P, Montoro JB. et al. Inhaled tobramycin in non-cystic fibrosis patients with bronchiectasis and chronic bronchial infection with Pseudomonas aeruginosa. Ann Pharmacother 2005; 39: 39-44
• 397 Serisier DJ, Bilton D, De Soyza A. et al. Inhaled, dual release liposomal ciprofloxacin in non-cystic fibrosis bronchiectasis (ORBIT-2): a randomised, double-blind, placebo-controlled trial. Thorax 2013; 68: 812-817
• 398 Barker AF, OʼDonnell AE, Flume P. et al. Aztreonam for inhalation solution in patients with non-cystic fibrosis bronchiectasis (AIR-BX1 and AIR-BX2): two randomised double-blind, placebo-controlled phase 3 trials. Lancet Respir Med 2014; 2: 738-749
• 399 Terpstra LC, Altenburg J, Bronsveld I. et al. Effects of long-term tobramycin inhalation solution (TIS) once daily on exacerbation rate in patients with non-cystic fibrosis bronchiectasis. Respir Res 2022; 23: 330
• 400 Guan WJ, Xu JF, Luo H. et al. A Double-Blind Randomized Placebo-Controlled Phase 3 Trial of Tobramycin Inhalation Solution in Adults With Bronchiectasis With Pseudomonas aeruginosa Infection. Chest 2023; 163: 64-76
• 401 Wilson R, Welte T, Polverino E. et al. Ciprofloxacin dry powder for inhalation in non-cystic fibrosis bronchiectasis: a phase II randomised study. Eur Respir J 2013; 41: 1107-1115
• 402 Sibila O, Laserna E, Shoemark A. et al. Heterogeneity of treatment response in bronchiectasis clinical trials. Eur Respir J 2022; 59: 2100777
• 403 Brodt AM, Stovold E, Zhang L. Inhaled antibiotics for stable non-cystic fibrosis bronchiectasis: a systematic review. Eur Respir J 2014; 44: 382-393
• 404 Xu L, Zhang F, Du S. et al. Inhaled antibiotics in non-cystic fibrosis bronchiectasis: A meta-analysis. Pharmazie 2016; 71: 491-498
• 405 Yang JW, Fan LC, Lu HW. et al. Efficacy and safety of long-term inhaled antibiotic for patients with noncystic fibrosis bronchiectasis: a meta-analysis. Clin Respir J 2016; 10: 731-739
• 406 Lim JU, Hong SW, Ko JH. Efficacy of inhaled ciprofloxacin agents for the treatment of bronchiectasis: a systematic review and meta-analysis of randomized controlled trials. Ther Adv Respir Dis 2019; 13: 1753466619875930
• 407 Xu MJ, Dai B. Inhaled antibiotics therapy for stable non-cystic fibrosis bronchiectasis: a meta-analysis. Ther Adv Respir Dis 2020; 14: 1753466620936866
• 408 Tejada S, Campogiani L, Sole-Lleonart C. et al. Inhaled antibiotics for treatment of adults with non-cystic fibrosis bronchiectasis: A systematic review and meta-analysis. Eur J Intern Med 2021; 90: 77-88
• 409 Sangiovanni S, Morales EI, Fernandez-Trujillo L. Inhaled tobramycin for chronic infection with pseudomonas aeruginosa in non-cystic fibrosis bronchiectasis: A systematic review and meta-analysis. Respir Med 2021; 176: 106283
• 410 Wang S, Zhang A, Yao X. Meta-analysis of efficacy and safety of inhaled ciprofloxacin in non-cystic fibrosis bronchiectasis patients. Intern Med J 2021; 51: 1505-1512
• 411 Tejada S, Ramirez-Estrada S, Forero CG. et al. Safety and Efficacy of Devices Delivering Inhaled Antibiotics among Adults with Non-Cystic Fibrosis Bronchiectasis: A Systematic Review and a Network Meta-Analysis. Antibiotics (Basel) 2022; 11: 275
• 412 Chalmers JD, Smith MP, McHugh BJ. et al. Short- and long-term antibiotic treatment reduces airway and systemic inflammation in non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med 2012; 186: 657-665
• 413 Sibila O, Laserna E, Shoemark A. et al. Airway Bacterial Load and Inhaled Antibiotic Response in Bronchiectasis. Am J Respir Crit Care Med 2019; 200: 33-41
• 414 Chalmers JD, Cipolla D, Thompson B. et al. Changes in respiratory symptoms during 48-week treatment with ARD-3150 (inhaled liposomal ciprofloxacin) in bronchiectasis: results from the ORBIT-3 and -4 studies. Eur Respir J 2020; 56: 2000110
• 415 Haworth CS, Shteinberg M, Winthrop K. et al. Inhaled colistimethate sodium in patients with bronchiectasis and Pseudomonas aeruginosa infection: results of PROMIS-I and PROMIS-II, two randomised, double-blind, placebo-controlled phase 3 trials assessing safety and efficacy over 12 months. Lancet Respir Med 2024; 12: 787-798
• 416 Nadig TR, Flume PA. Aerosolized Antibiotics for Patients with Bronchiectasis. Am J Respir Crit Care Med 2016; 193: 808-810
• 417 Ilowite JS, Gorvoy JD, Smaldone GC. Quantitative deposition of aerosolized gentamicin in cystic fibrosis. Am Rev Respir Dis 1987; 136: 1445-1449
• 418 Mukhopadhyay S, Staddon GE, Eastman C. et al. The quantitative distribution of nebulized antibiotic in the lung in cystic fibrosis. Respir Med 1994; 88: 203-211
• 419 Klinger-Strobel M, Lautenschlager C, Fischer D. et al. Aspects of pulmonary drug delivery strategies for infections in cystic fibrosis -- where do we stand?. Expert Opin Drug Deliv 2015; 12: 1351-1374
• 420 Morosini MI, Garcia-Castillo M, Loza E. et al. Breakpoints for predicting Pseudomonas aeruginosa susceptibility to inhaled tobramycin in cystic fibrosis patients: use of high-range Etest strips. J Clin Microbiol 2005; 43: 4480-4485
• 421 Gorham J, Taccone FS, Hites M. How to Use Nebulized Antibiotics in Severe Respiratory Infections. Antibiotics-Basel 2023; 12: 267
• 422 Currie DC, Garbett ND, Chan KL. et al. Double-Blind Randomized Study of Prolonged Higher-Dose Oral Amoxycillin in Purulent Bronchiectasis. Q J Med 1990; 76: 799-816
• 423 Wu Q, Shen W, Cheng H. et al. Long-term macrolides for non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis. Respirology 2014; 19: 321-329
• 424 Shi ZL, Peng H, Hu XW. et al. Effectiveness and safety of macrolides in bronchiectasis patients: a meta-analysis and systematic review. Pulm Pharmacol Ther 2014; 28: 171-178
• 425 Gao YH, Guan WJ, Xu G. et al. Macrolide therapy in adults and children with non-cystic fibrosis bronchiectasis: a systematic review and meta-analysis. PLoS One 2014; 9: e90047
• 426 Zhuo GY, He Q, Xiang-Lian L. et al. Prolonged treatment with macrolides in adult patients with non-cystic fibrosis bronchiectasis: meta-analysis of randomized controlled trials. Pulm Pharmacol Ther 2014; 29: 80-88
• 427 Wang D, Fu W, Dai J. Meta-analysis of macrolide maintenance therapy for prevention of disease exacerbations in patients with noncystic fibrosis bronchiectasis. Medicine (Baltimore) 2019; 98: e15285
• 428 Kobbernagel HE, Buchvald FF, Haarman EG. et al. Efficacy and safety of azithromycin maintenance therapy in primary ciliary dyskinesia (BESTCILIA): a multicentre, double-blind, randomised, placebo-controlled phase 3 trial. Lancet Respir Med 2020; 8: 493-505
• 429 Ray WA, Murray KT, Hall K. et al. Azithromycin and the risk of cardiovascular death. N Engl J Med 2012; 366: 1881-1890
• 430 Albert RK, Connett J, Bailey WC. et al. Azithromycin for prevention of exacerbations of COPD. N Engl J Med 2011; 365: 689-698
• 431 Kanoh S, Rubin BK. Mechanisms of action and clinical application of macrolides as immunomodulatory medications. Clin Microbiol Rev 2010; 23: 590-615
• 432 Chadha J, Harjai K, Chhibber S. Revisiting the virulence hallmarks of Pseudomonas aeruginosa: a chronicle through the perspective of quorum sensing. Environ Microbiol 2022; 24: 2630-2656
• 433 Hamed MM, Abdelsamie AS, Rox K. et al. Towards Translation of PqsR Inverse Agonists: From In Vitro Efficacy Optimization to In Vivo Proof-of-Principle. Adv Sci (Weinh) 2023; 10: e2204443
• 434 Mandal P, Sidhu MK, Donaldson LS. et al. Eight-weekly intravenous antibiotics is beneficial in severe bronchiectasis. QJM 2013; 106: 27-33
• 435 Hnin K, Nguyen C, Carson KV. et al. Prolonged antibiotics for non-cystic fibrosis bronchiectasis in children and adults. Cochrane Db Syst Rev 2015;
• 436 Rucker RW, Harrison GM. Outpatient intravenous medications in the management of cystic fibrosis. Pediatrics 1974; 54: 358-360
• 437 Bedi P, Sidhu MK, Donaldson LS. et al. A prospective cohort study of the use of domiciliary intravenous antibiotics in bronchiectasis. NPJ Prim Care Respir Med 2014; 24: 14090
• 438 López-Cortés LE, Ayerbe-García R, Carrasco-Hernández L. et al. Outpatient Parenteral Antimicrobial Treatment for Non-Cystic Fibrosis Bronchiectasis Exacerbations: A Prospective Multicentre Observational Cohort Study. Respiration 2019; 98: 294-300
• 439 Lenney W, Edenborough F, Kho P. et al. Lung deposition of inhaled tobramycin with eFlow rapid/LC Plus jet nebuliser in healthy and cystic fibrosis subjects. J Cyst Fibros 2011; 10: 9-14
• 440 Fokkens WJ, Lund VJ, Hopkins C. et al. European Position Paper on Rhinosinusitis and Nasal Polyps 2020. Rhinology 2020; 58: 1-464
• 441 Stuck BA, Popert U, Beule A. et al. S2k-Leitlinie Rhinosinusitis. https://www.awmf.org/leitlinien/detail/ll/053-012.html (abgerufen am: 06.06.2022)
• 442 Guilemany JM, Alobid I, Angrill J. et al. The impact of bronchiectasis associated to sinonasal disease on quality of life. Respir Med 2006; 100: 1997-2003
• 443 Guilemany JM, Angrill J, Alobid I. et al. United airways again: high prevalence of rhinosinusitis and nasal polyps in bronchiectasis. Allergy 2009; 64: 790-797
• 444 Guilemany JM, Angrill J, Alobid I. et al. United airways: the impact of chronic rhinosinusitis and nasal polyps in bronchiectasic patientʼs quality of life. Allergy 2009; 64: 1524-1529
• 445 Shteinberg M, Nassrallah N, Jrbashyan J. et al. Upper airway involvement in bronchiectasis is marked by early onset and allergic features. ERJ Open Res 2018; 4: 00115-2017
• 446 Yang X, Xu Y, Jin J. et al. Chronic rhinosinusitis is associated with higher prevalence and severity of bronchiectasis in patients with COPD. Int J Chron Obstruct Pulmon Dis 2017; 12: 655-662
• 447 Sheng H, Yao X, Wang X. et al. Prevalence and clinical implications of bronchiectasis in patients with overlapping asthma and chronic rhinosinusitis: a single-center prospective study. BMC Pulm Med 2021; 21: 211
• 448 Pfaar O, Beule AG, Laudien M. et al. [Treatment of chronic rhinosinusitis with nasal polyps (CRSwNP) with monoclonal antibodies (biologics): S2k guideline of the German Society of Oto-Rhino-Laryngology, Head and Neck Surgery (DGHNO-KHC), and the German College of General Practitioners and Family Physicians (DEGAM)]. HNO 2023; 71: 256-263
• 449 Mainz JG, Schadlich K, Schien C. et al. Sinonasal inhalation of tobramycin vibrating aerosol in cystic fibrosis patients with upper airway Pseudomonas aeruginosa colonization: results of a randomized, double-blind, placebo-controlled pilot study. Drug Des Devel Ther 2014; 8: 209-217
• 450 Mainz JG, Schien C, Schiller I. et al. Sinonasal inhalation of dornase alfa administered by vibrating aerosol to cystic fibrosis patients: a double-blind placebo-controlled cross-over trial. J Cyst Fibros 2014; 13: 461-470
• 451 Mainz JG, Schumacher U, Schadlich K. et al. Sino nasal inhalation of isotonic versus hypertonic saline (6.0%) in CF patients with chronic rhinosinusitis – Results of a multicenter, prospective, randomized, double-blind, controlled trial. J Cyst Fibros 2016; 15: e57-e66
• 452 Albrecht T, Beule AG, Hildenbrand T. et al. Cross-cultural adaptation and validation of the 22-item sinonasal outcome test (SNOT-22) in German-speaking patients: a prospective, multicenter cohort study. Eur Arch Otorhinolaryngol 2022; 279: 2433-2439
• 453 Klimek L, Forster-Ruhrmann U, Becker S. et al. Positionspapier: Anwendung von Biologika bei chronischer Rhinosinusitis mit Polyposis nasi (CRSwNP) im deutschen Gesundheitssystem – Empfehlungen des Ärzteverbandes Deutscher Allergologen (AeDA) und der AGs Klinische Immunologie, Allergologie und Umweltmedizin und Rhinologie und Rhinochirurgie der Deutschen Gesellschaft fur HNO-Heilkunde, Kopf- und Halschirurgie (DGHNOKHC). Laryngorhinootologie 2020; 99: 511-527
• 454 Bachert C, Han JK, Desrosiers M. et al. Efficacy and safety of dupilumab in patients with severe chronic rhinosinusitis with nasal polyps (LIBERTY NP SINUS-24 and LIBERTY NP SINUS-52): results from two multicentre, randomised, double-blind, placebo-controlled, parallel-group phase 3 trials. Lancet 2019; 394: 1638-1650
• 455 Gevaert P, Omachi TA, Corren J. et al. Efficacy and safety of omalizumab in nasal polyposis: 2 randomized phase 3 trials. J Allergy Clin Immunol 2020; 146: 595-605
• 456 Kanjanaumporn J, Hwang PH. Effect of Endoscopic Sinus Surgery on Bronchiectasis Patients With Chronic Rhinosinusitis. Am J Rhinol Allergy 2018; 32: 432-439
• 457 Wang Y, Yang HB. Effects of functional endoscopic sinus surgery on the treatment of bronchiectasis combined with chronic rhino-sinusitis. Acta Otolaryngol 2016; 136: 860-863
• 458 Clofent D, Alvarez A, Traversi L. et al. Comorbidities and mortality risk factors for patients with bronchiectasis. Expert Rev Respir Med 2021; 15: 623-634
• 459 Onen ZP, Gulbay BE, Sen E. et al. Analysis of the factors related to mortality in patients with bronchiectasis. Resp Med 2007; 101: 1390-1397
• 460 Loebinger MR, Wells AU, Hansell DM. et al. Mortality in bronchiectasis: a long-term study assessing the factors influencing survival. Eur Respir J 2009; 34: 843-849
• 461 Goeminne PC, Nawrot TS, Ruttens D. et al. Mortality in non-cystic fibrosis bronchiectasis: a prospective cohort analysis. Respir Med 2014; 108: 287-296
• 462 Nowinski A, Stachyra K, Szybinska M. et al. The influence of comorbidities on mortality in bronchiectasis: A prospective, observational study. Adv Clin Exp Med 2021; 30: 1315-1321
• 463 Navaratnam V, Millett ERC, Hurst JR. et al. Bronchiectasis and the risk of cardiovascular disease: a population-based study. Thorax 2017; 72: 161-166
• 464 Ozgun Niksarlioglu EY, Ozkan G, Gunluoglu G. et al. Factors related to depression and anxiety in adults with bronchiectasis. Neuropsychiatr Dis Treat 2016; 12: 3005-3010
• 465 Dudgeon EK, Crichton M, Chalmers JD. "The missing ingredient": the patient perspective of health related quality of life in bronchiectasis: a qualitative study. BMC Pulm Med 2018; 18: 81
• 466 Kelly C, Grundy S, Lynes D. et al. Self-management for bronchiectasis. Cochrane Database Syst Rev 2018; 2: CD012528
• 467 Schechter MS, Ostrenga JS, Fink AK. et al. Decreased survival in cystic fibrosis patients with a positive screen for depression. J Cyst Fibros 2021; 20: 120-126
• 468 Colomo N, Olveira C, Hernandez-Pedrosa J. et al. Validity of Self-rating Screening Scales for the Diagnosis of Depression and Anxiety in Adult Patients With Bronchiectasis. Arch Bronconeumol (Engl Ed) 2021; 57: 179-185
• 469 Olveira C, Olveira G, Gaspar I. et al. Depression and anxiety symptoms in bronchiectasis: associations with health-related quality of life. Qual Life Res 2013; 22: 597-605
• 470 Quittner AL, Abbott J, Georgiopoulos AM. et al. International Committee on Mental Health in Cystic Fibrosis: Cystic Fibrosis Foundation and European Cystic Fibrosis Society consensus statements for screening and treating depression and anxiety. Thorax 2016; 71: 26-34
• 471 Martinez-Garcia MA, Miravitlles M. Bronchiectasis in COPD patients: more than a comorbidity?. Int J Chronic Obstr 2017; 12: 1401-1411
• 472 Martinez-Garcia MA, Miravitlles M. Bronchiectasis in COPD patients: more than a comorbidity? (vol 12, pg 1401, 2017). Int J Chronic Obstr 2019; 14: 245-245
• 473 Traversi L, Miravitlles M, Martinez-Garcia MA. et al. ROSE: radiology, obstruction, symptoms and exposure – a Delphi consensus definition of the association of COPD and bronchiectasis by the EMBARC Airways Working Group. ERJ Open Res 2021; 7: 00399-2021
• 474 Du QX, Jin JM, Liu XF. et al. Bronchiectasis as a Comorbidity of Chronic Obstructive Pulmonary Disease: A Systematic Review and Meta-Analysis. Plos One 2016; 11: e0150532
• 475 Kahnert K, Jorres RA, Kauczor HU. et al. Relationship between clinical and radiological signs of bronchiectasis in COPD patients: Results from COSYCONET. Resp Med 2020; 172: 106117
• 476 Dimakou K, Gousiou A, Toumbis M. et al. Investigation of bronchiectasis in severe uncontrolled asthma. Clin Respir J 2018; 12: 1212-1218
• 477 Sanchez-Munoz G, Lopez-de-Andres A, Jimenez-Garcia R. et al. Trend from 2001 to 2015 in the prevalence of bronchiectasis among patients hospitalized for asthma and effect of bronchiectasis on the in-hospital mortality. J Asthma 2021; 58: 1067-1076
• 478 Martinez-Garcia MA, Rosa Carrillo D de la, Soler-Cataluna JJ. et al. Prognostic value of bronchiectasis in patients with moderate-to-severe chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2013; 187: 823-831
• 479 Polverino E, De Soyza A, Dimakou K. et al. The Association Between Bronchiectasis and Chronic Obstructive Pulmonary Disease: Data from the European Bronchiectasis Registry (EMBARC). Am J Respir Crit Care Med 2024;
• 480 Polverino E, Rosales-Mayor E, Benegas M. et al. Pneumonic and non-pneumonic exacerbations in bronchiectasis: Clinical and microbiological differences. J Infect 2018; 77: 99-106
• 481 Ewig S, Kolditz M, Pletz M. et al. [Management of Adult Community-Acquired Pneumonia and Prevention – Update 2021 – Guideline of the German Respiratory Society (DGP), the Paul-Ehrlich-Society for Chemotherapy (PEG), the German Society for Infectious Diseases (DGI), the German Society of Medical Intensive Care and Emergency Medicine (DGIIN), the German Viological Society (DGV), the Competence Network CAPNETZ, the German College of General Practitioneers and Family Physicians (DEGAM), the German Society for Geriatric Medicine (DGG), the German Palliative Society (DGP), the Austrian Society of Pneumology Society (OGP), the Austrian Society for Infectious and Tropical Diseases (OGIT), the Swiss Respiratory Society (SGP) and the Swiss Society for Infectious Diseases Society (SSI)]. Pneumologie 2021; 75: 665-729
• 482 Brunkhorst FM, Weigand MA, Pletz M. et al. [S3 Guideline Sepsis-prevention, diagnosis, therapy, and aftercare : Long version]. Med Klin Intensivmed Notfmed 2020; 115: 37-109
• 483 Bilton D, Henig N, Morrissey B. et al. Addition of inhaled tobramycin to ciprofloxacin for acute exacerbations of Pseudomonas aeruginosa infection in adult bronchiectasis. Chest 2006; 130: 1503-1510
• 484 Bedi P, Cartlidge MK, Zhang Y. et al. Feasibility of shortening intravenous antibiotic therapy for bronchiectasis based on bacterial load: a proof-of-concept randomised controlled trial. Eur Respir J 2021; 58: 2004388
• 485 Hadda V, Chawla G, Tiwari P. et al. Noninvasive Ventilation for Acute Respiratory Failure due to Noncystic Fibrosis Bronchiectasis. Indian J Crit Care Med 2018; 22: 326-331
• 486 Alzeer AH, Masood M, Basha SJ. et al. Survival of bronchiectatic patients with respiratory failure in ICU. BMC Pulm Med 2007; 7: 17
• 487 Agarwal R, Gupta R, Aggarwal AN. et al. Noninvasive positive pressure ventilation in acute respiratory failure due to COPD vs other causes: effectiveness and predictors of failure in a respiratory ICU in North India. Int J Chron Obstruct Pulmon Dis 2008; 3: 737-743
• 488 Bello G, De Pascale G, Antonelli M. Noninvasive Ventilation. Clin Chest Med 2016; 37: 711-721
• 489 Amati F, Gramegna A, Contarini M. et al. Management of primary ciliary dyskinesia: current practice and future perspectives. Eur Respir Monogr 2018; 81: 353-370
• 490 Garpestad E, Brennan J, Hill NS. Noninvasive ventilation for critical care. Chest 2007; 132: 711-720
• 491 Phua J, Ang YL, See KC. et al. Noninvasive and invasive ventilation in acute respiratory failure associated with bronchiectasis. Intensive Care Med 2010; 36: 638-647
• 492 Scala R, Pisani L. Noninvasive ventilation in acute respiratory failure: which recipe for success?. Eur Respir Rev 2018; 27: 180029
• 493 Westhoff M, Neumann P, Geiseler J. et al. [Non-invasive Mechanical Ventilation in Acute Respiratory Failure. Clinical Practice Guidelines – on behalf of the German Society of Pneumology and Ventilatory Medicine]. Pneumologie 2023;
• 494 Dimakou K, Triantafillidou C, Toumbis M. et al. Non CF-bronchiectasis: Aetiologic approach, clinical, radiological, microbiological and functional profile in 277 patients. Respir Med 2016; 116: 1-7
• 495 King P, Holdsworth S, Freezer N. et al. Bronchiectasis. Intern Med J 2006; 36: 729-737
• 496 Flume PA, Yankaskas JR, Ebeling M. et al. Massive hemoptysis in cystic fibrosis. Chest 2005; 128: 729-738
• 497 Jean-Baptiste E. Clinical assessment and management of massive hemoptysis. Crit Care Med 2001; 29: 1098-1098
• 498 Lee MK, Kim SH, Yong SJ. et al. Moderate hemoptysis: recurrent hemoptysis and mortality according to bronchial artery embolization. Clin Respir J 2015; 9: 53-64
• 499 Gopinath B, Mishra PR, Aggarwal P. et al. Nebulized vs IV Tranexamic Acid for Hemoptysis: A Pilot Randomized Controlled Trial. Chest 2023; 163: 1176-1184
• 500 Takeda K, Kawashima M, Masuda K. et al. Long-Term Outcomes of Bronchial Artery Embolization for Patients with Non-Mycobacterial Non-Fungal Infection Bronchiectasis. Respiration 2020; 99: 961-969
• 501 Mondoni M, Carlucci P, Cipolla G. et al. Long-term prognostic outcomes in patients with haemoptysis. Respir Res 2021; 22: 219
• 502 Maleux G, Matton T, Laenen A. et al. Safety and Efficacy of Repeat Embolization for Recurrent Hemoptysis: A 16-Year Retrospective Study Including 223 Patients. J Vasc Interv Radiol 2018; 29: 502-509
• 503 Lee JH, Kwon SY, Yoon HI. et al. Haemoptysis due to chronic tuberculosis vs. bronchiectasis: comparison of long-term outcome of arterial embolisation. Int J Tuberc Lung D 2007; 11: 781-787
• 504 Jang EJ, Lee CH, Yoon HI. et al. Association between inhaler use and risk of haemoptysis in patients with non-cystic fibrosis bronchiectasis. Respirology 2015; 20: 1213-1221
• 505 Bock R, Schultz K. Die vier Wege zur Rehabilitation. PneumoJournal 2017; 2: 4-6
• 506 Kelly CA, Tsang A, Lynes D. et al. ‘Itʼs not one size fits all’: a qualitative study of patients' and healthcare professionalsʼ views of self-management for bronchiectasis. BMJ Open Respir Res 2021; 8: e000862
• 507 Tsang A, Lynes D, McKenzie H. et al. Self-management programmes for adult patients with bronchiectasis: a systematic review and realist synthesis. Disabil Rehabil 2022; 44: 6939-6948
• 508 Pasteur MC, Helliwell SM, Houghton SJ. et al. An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000; 162: 1277-1284
• 509 Transitionsmedizin Gfr. S3-Leitlinie: Transition von der Pädiatrie in die Erwachsenenmedizin; Langversion 1.1 vom 22.04.2021 (AWMF-Registernummer: 186-001). https://www.awmf.org/leitlinien/detail/ll/186-001.html Zugriff: 05.06.2022
• 510 Kuehni CE, Goutaki M, Rubbo B. et al. Management of primary ciliary dyskinesia: current practice and future perspectives. Eur Respir Monogr 2018; 81: 282-299
• 511 Moss R, Farrant B, Byrnes CA. Transitioning from paediatric to adult services with cystic fibrosis or bronchiectasis: What is the impact on engagement and health outcomes?. J Paediatr Child Health 2021; 57: 548-553
• 512 Castellani C, Duff AJA, Bell SC. et al. ECFS best practice guidelines: the 2018 revision. J Cyst Fibros 2018; 17: 153-178
• 513 Elborn JS, Bell SC, Madge SL. et al. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis. Eur Respir J 2016; 47: 420-428
• 514 Middleton PG, Gade EJ, Aguilera C. et al. ERS/TSANZ Task Force Statement on the management of reproduction and pregnancy in women with airways diseases. Eur Respir J 2020; 55: 1901208
• 515 Fuchs HJ, Borowitz DS, Christiansen DH. et al. Effect of aerosolized recombinant human DNase on exacerbations of respiratory symptoms and on pulmonary function in patients with cystic fibrosis. The Pulmozyme Study Group. N Engl J Med 1994; 331: 637-642
• 516 Koletzko B, Cremer M, Flothkotter M. et al. Diet and Lifestyle Before and During Pregnancy – Practical Recommendations of the Germany-wide Healthy Start – Young Family Network. Geburtshilfe Frauenheilkd 2018; 78: 1262-1282
• 517 Munro NC, Currie DC, Lindsay KS. et al. Fertility in men with primary ciliary dyskinesia presenting with respiratory infection. Thorax 1994; 49: 684-687
• 518 Sha YW, Ding L, Li P. Management of primary ciliary dyskinesia/Kartagenerʼs syndrome in infertile male patients and current progress in defining the underlying genetic mechanism. Asian J Androl 2014; 16: 101-106
• 519 Naehrlich L, Stuhrmann-Spangenberg M, Barben J. et al. S2-Konsensus-Leitlinie „Diagnose der Mukoviszidose“ (AWMF 026-023) unter Federführung der Gesellschaft für Pädiatrischen Pneumologie. https://www.awmf.org/leitlinien/detail/ll/026-023.html (abgerufen am: 05.06.2022)
• 520 Deutsche Gesellschaft für Humangenetik e. V. und Berufsverband Deutscher Humangenetiker e. V. S2k-Leitlinie Humangenetische Diagnostik und Genetische Beratung. medgen 2018; 30: 469-522
• 521 Ashcroft A, Chapman SJ, Mackillop L. The outcome of pregnancy in women with cystic fibrosis: a UK population-based descriptive study. BJOG 2020; 127: 1696-1703
• 522 Reynaud Q, Rousset Jablonski C, Poupon-Bourdy S. et al. Pregnancy outcome in women with cystic fibrosis and poor pulmonary function. J Cyst Fibros 2020; 19: 80-83
• 523 Deutsche Gesellschaft für Gynäkologie und Geburtshilfe e. V. und Deutsche Gesellschaft für Hebammenwissenschaft e. V.. Die vaginale Geburt am Termin. https://www.awmf.org/leitlinien/detail/ll/015-083.html (abgerufen am: 05.06.2022)
• 524 Elkus R, Popovich Jr J. Respiratory physiology in pregnancy. Clin Chest Med 1992; 13: 555-565
• 525 Edenborough FP, Borgo G, Knoop C. et al. Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros 2008; 7 (Suppl. 01) S2-32
• 526 Kroon M, Akkerman-Nijland AM, Rottier BL. et al. Drugs during pregnancy and breast feeding in women diagnosed with Cystic Fibrosis – An update. J Cyst Fibros 2018; 17: 17-25
• 527 Statistisches Bundesamt (Destatis). Drei von vier Müttern in Deutschland waren 2019 erwerbstätig. https://www.destatis.de/DE/Presse/Pressemitteilungen/2021/03/PD21_N017_13.html (abgerufen am: 05.06.2021)
• 528 Mc Veigh C, Reid J, Larkin P. et al. Palliative care for people with non-malignant respiratory disease and their carers: a review of the current evidence. J Res Nurs 2019; 24: 420-430
• 529 Simon ST, Pralong A, Radbruch L. et al. The Palliative Care of Patients With Incurable Cancer. Dtsch Arztebl Int 2020; 116: 108-115
• 530 Mc Veigh C, Reid J, Hudson P. et al. The experiences of palliative care health service provision for people with non-malignant respiratory disease and their caregivers: an all-Ireland study. J Adv Nurs 2014; 70: 687-697
• 531 Neo HY, Yap CW, Teo LM. et al. Palliative Rehabilitation Improves Health Care Utilization and Function in Frail Older Adults with Chronic Lung Diseases. J Am Med Dir Assoc 2021; 22: 2478-2485 e2471
• 532 Reipas KM, Grossman DL, Lock K. et al. Examining the Characteristics of Patients With Non-Malignant Lung Disease at the Time of Referral to An Inter-Professional Supportive Care Clinic. Am J Hosp Palliat Care 2021; 38: 1329-1335
• 533 Aksamit TR, Emery EJ, Basavaraj A. et al. The 6th World Bronchiectasis and Nontuberculous Mycobacteria Conference Abstract Presentations. Chronic Obstr Pulm Dis 2023; 10: 450-516
• 534 Chalmers JD, Aksamit T, Aliberti S. et al. World Bronchiectasis Day 2022. Eur Respir J 2022; 59: 2201249
• 535 Chalmers JD, Aliberti S, Altenburg J. et al. Transforming clinical research and science in bronchiectasis: EMBARC3, a European Respiratory Society Clinical Research Collaboration. Eur Respir J 2023; 61: 2300769
• 536 Dhar R, Singh S, Talwar D. et al. Bronchiectasis in India: results from the European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) and Respiratory Research Network of India Registry. Lancet Glob Health 2019; 7: e1269-e1279
• 537 Schaupp L, Addante A, Voller M. et al. Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on sputum viscoelastic properties, airway infection and inflammation in patients with cystic fibrosis. Eur Respir J 2023; 62: 2202153
• 538 Shoemark A, Griffin H, Wheway G. et al. Genome sequencing reveals underdiagnosis of primary ciliary dyskinesia in bronchiectasis. Eur Respir J 2022; 60: 2200176
• 539 Simon S, Joean O, Welte T. et al. The role of vaccination in COPD: influenza, SARS-CoV-2, pneumococcus, pertussis, RSV and varicella zoster virus. Eur Respir Rev 2023; 32: 230034
• 540 Lommatzsch M, Buhl R, Canonica GW. et al. Pioneering a paradigm shift in asthma management: remission as a treatment goal. Lancet Respir Med 2024; 12: 96-99