Semin Respir Crit Care Med 2021; 42(04): 513-524
DOI: 10.1055/s-0041-1730892
Review Article

Diagnosis and Initial Investigation of Bronchiectasis

Francesco Amati
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Edoardo Simonetta
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
2   Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
,
Tommaso Pilocane
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
2   Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
,
Andrea Gramegna
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
2   Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
,
Pieter Goeminne
3   Department of Respiratory Medicine, AZ Nikolaas, Sint-Niklaas, Belgium
,
Martina Oriano
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
2   Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
,
Sergi Pascual-Guardia
4   Department of Respiratory Medicine, Hospital del Mar (PSMAR)—IMIM, Barcelona, Spain
5   School of Health and Life Sciences, Universitat Pompeu Fabra, Barcelona, Spain
6   CIBER, Área de Enfermedades Respiratorias (CIBERES), ISCIII, Spain
,
Marco Mantero
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
2   Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
,
Antonio Voza
7   Emergency Department, Humanitas Clinical and Research Center, IRCCS, Milan, Italy
,
Martina Santambrogio
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
,
Francesco Blasi
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
2   Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
,
Stefano Aliberti
1   Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy
2   Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy
› Author Affiliations

Abstract

Bronchiectasis refers to both the name of a disease and a single radiological appearance that may, or may not, be associated with disease. As chronic respiratory disease, bronchiectasis is characterized by a variable range of signs and symptoms that may overlap with other chronic respiratory conditions. The proper identification of bronchiectasis as a disease in both primary and secondary care is of paramount importance. However, a standardized definition of radiologically and clinically significant bronchiectasis is still missing. Disease heterogeneity is a hallmark of bronchiectasis and applies not only to radiological features and clinical manifestations but also to other aspects of the disease, including the etiological and microbiological diagnosis as well as the evaluation of pulmonary function. Although the guidelines suggest a “minimum bundle” of tests, the diagnostic approach to bronchiectasis is challenging and may be driven by the “treatable traits” approach based on endotypes and biological characteristics. A broad spectrum of diagnostic tests could be used to investigate the etiology of bronchiectasis as well as other pulmonary, extrapulmonary, and environmental traits. Individualizing bronchiectasis workup according to the site of care (e.g., primary, secondary, and tertiary care) could help optimize patients' management and reduce healthcare costs.



Publication History

Article published online:
14 July 2021

© 2021. Thieme. All rights reserved.

Thieme Medical Publishers, Inc.
333 Seventh Avenue, 18th Floor, New York, NY 10001, USA

 
  • References

  • 1 Flume PA, Chalmers JD, Olivier KN. Advances in bronchiectasis: endotyping, genetics, microbiome, and disease heterogeneity. Lancet 2018; 392 (10150): 880-890
  • 2 Chalmers JD, Sethi S. Raising awareness of bronchiectasis in primary care: overview of diagnosis and management strategies in adults. NPJ Prim Care Respir Med 2017; 27 (01) 18
  • 3 Radovanovic D, Santus P, Blasi F. et al. A comprehensive approach to lung function in bronchiectasis. Respir Med 2018; 145: 120-129
  • 4 Gao YH, Guan WJ, Liu SX. et al. Aetiology of bronchiectasis in adults: a systematic literature review. Respirology 2016; 21 (08) 1376-1383
  • 5 Amati F, Simonetta E, Gramegna A. et al. The biology of pulmonary exacerbations in bronchiectasis. Eur Respir Rev 2019; 28 (154) 190055
  • 6 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 (12) 969-979
  • 7 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 (01) 186-193
  • 8 Ringshausen FC, de Roux A, Diel R, Hohmann D, Welte T, Rademacher J. Bronchiectasis in Germany: a population-based estimation of disease prevalence. Eur Respir J 2015; 46 (06) 1805-1807
  • 9 Monteagudo M, Rodríguez-Blanco T, Barrecheguren M, Simonet P, Miravitlles M. Prevalence and incidence of bronchiectasis in Catalonia, Spain: a population-based study. Respir Med 2016; 121: 26-31
  • 10 Aliberti S, Sotgiu G, Lapi F, Gramegna A, Cricelli C, Blasi F. Prevalence and incidence of bronchiectasis in Italy. BMC Pulm Med 2020; 20 (01) 15
  • 11 Weycker D, Hansen GL, Seifer FD. Prevalence and incidence of noncystic fibrosis bronchiectasis among US adults in 2013. Chron Respir Dis 2017; 14 (04) 377-384
  • 12 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 (06) 1900499
  • 13 Chandrasekaran R, Mac Aogáin M, Chalmers JD, Elborn SJ, Chotirmall SH. Geographic variation in the aetiology, epidemiology and microbiology of bronchiectasis. BMC Pulm Med 2018; 18 (01) 83
  • 14 Aksamit TR, O'Donnell AE, Barker A. et al; Bronchiectasis Research Registry Consortium. Adult patients with bronchiectasis: a first look at the US Bronchiectasis Research Registry. Chest 2017; 151 (05) 982-992
  • 15 Gramegna A, Amati F, Chalmers JD, Elborn SJ, Aliberti S, Blasi F. How to identify causes and predisposing factors in bronchiectasis. In: Chalmers J, Polverino E, Aliberti S. eds. Bronchiectasis. Cham, Switzerland: Springer; 2018
  • 16 Seitz AE, Olivier KN, Adjemian J, Holland SM, Prevots DR. Trends in bronchiectasis among Medicare beneficiaries in the United States, 2000 to 2007. Chest 2012; 142 (02) 432-439
  • 17 de la Rosa Carrillo D, Navarro Rolon A, Girón Moreno RM. et al. Cost of hospitalizations due to exacerbation in patients with non-cystic fibrosis bronchiectasis. Respiration 2018; 96 (05) 406-416
  • 18 Diel R, Chalmers JD, Rabe KF, Nienhaus A, Loddenkemper R, Ringshausen FC. Economic burden of bronchiectasis in Germany. Eur Respir J 2019; 53 (02) 1802033
  • 19 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 (09) e1269-e1279
  • 20 Aliberti S, Polverino E, Chalmers JD. et al; EMBARC Clinical Research Collaboration. The European Multicentre Bronchiectasis Audit and Research Collaboration (EMBARC) ERS Clinical Research Collaboration. Eur Respir J 2018; 52 (05) 1802074
  • 21 Martínez-García MÁ, Máiz L, Olveira C. et al. Normativa sobre el tratamiento de las bronquiectasias en el adulto. Arch Bronconeumol 2018; 54 (02) 88-98
  • 22 Hill AT, Sullivan AL, Chalmers JD. et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax 2019; 74 (Suppl. 01) 1-69
  • 23 Polverino E, Goeminne PC, McDonnell MJ. et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J 2017; 50 (03) 1700629
  • 24 Chang AB, Bell SC, Torzillo PJ. et al; Extended Voting Group. Chronic suppurative lung disease and bronchiectasis in children and adults in Australia and New Zealand Thoracic Society of Australia and New Zealand guidelines. Med J Aust 2015; 202 (03) 130
  • 25 Turner RD, Bothamley GH. Chronic cough and a normal chest X-ray - a simple systematic approach to exclude common causes before referral to secondary care: a retrospective cohort study. NPJ Prim Care Respir Med 2016; 26: 15081
  • 26 Pasteur MC, Bilton D, Hill AT. British Thoracic Society Bronchiectasis non-CF Guideline Group. British Thoracic Society guideline for non-CF bronchiectasis. Thorax 2010; 65 (Suppl. 01) i1-i58
  • 27 Abdulmalak C, Cottenet J, Beltramo G. et al. Haemoptysis in adults: a 5-year study using the French nationwide hospital administrative database. Eur Respir J 2015; 46 (02) 503-511
  • 28 Anwar GA, McDonnell MJ, Worthy SA. et al. Phenotyping adults with non-cystic fibrosis bronchiectasis: a prospective observational cohort study. Respir Med 2013; 107 (07) 1001-1007
  • 29 Martínez-García M-A, de la Rosa Carrillo D, Soler-Cataluña J-J. et al. Prognostic value of bronchiectasis in patients with moderate-to-severe chronic obstructive pulmonary disease. Am J Respir Crit Care Med 2013; 187 (08) 823-831
  • 30 Kang HR, Choi GS, Park SJ. et al. The effects of bronchiectasis on asthma exacerbation. Tuberc Respir Dis (Seoul) 2014; 77 (05) 209-214
  • 31 Perez-Miranda J, Traversi L, Polverino E. Bronchiectasis in severe asthma: a distinct phenotype?. Curr Opin Pulm Med 2019; 25 (01) 71-78
  • 32 Global Initiative for Asthma. Difficult-to-treat & severe asthma in adolescent and adult patient, 2020. Accessed May 13, 2021 at: www.ginasthma.org
  • 33 Cohen M, Sahn SA. Bronchiectasis in systemic diseases. Chest 1999; 116 (04) 1063-1074
  • 34 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 (06) 1247-1254
  • 35 Santos VA, Tobón GJ, Cañas CA. Development of bronchiectasis during long-term rituximab treatment for rheumatoid arthritis. Adv Respir Med 2018; (epub ahead of print) DOI: 10.5603/ARM.a2018.0050.
  • 36 Wilczynska MM, Condliffe AM, McKeon DJ. Coexistence of bronchiectasis and rheumatoid arthritis: revisited. Respir Care 2013; 58 (04) 694-701
  • 37 Gathmann B, Mahlaoui N, Gérard L. et al; CEREDIH, Dutch WID, European Society for Immunodeficiencies Registry Working Party. Clinical picture and treatment of 2212 patients with common variable immunodeficiency. J Allergy Clin Immunol 2014; 134 (01) 116-126
  • 38 Maglione PJ, Overbey JR, Radigan L, Bagiella E, Cunningham-Rundles C. Pulmonary radiologic findings in common variable immunodeficiency: clinical and immunological correlations. Ann Allergy Asthma Immunol 2014; 113 (04) 452-459
  • 39 Wall LA, Wisner EL, Gipson KS, Sorensen RU. Bronchiectasis in primary antibody deficiencies: a multidisciplinary approach. Front Immunol 2020; 11: 522
  • 40 Hansell DM, Bankier AA, MacMahon H, McLoud TC, Müller NL, Remy J. Fleischner Society: glossary of terms for thoracic imaging. Radiology 2008; 246 (03) 697-722
  • 41 Meerburg JJ, Veerman GDM, Aliberti S, Tiddens HAWM. Diagnosis and quantification of bronchiectasis using computed tomography or magnetic resonance imaging: A systematic review. Respir Med 2020; 170: 105954
  • 42 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 (01) 1702052
  • 43 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 (01) 1702053
  • 44 Altenburg J, de Graaff CS, 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 2013; 309 (12) 1251-1259
  • 45 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 2013; 309 (12) 1260-1267
  • 46 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 (9842): 660-667
  • 47 Tiddens HAWM, Meerburg JJ, van der Eerden MM, Ciet P. The radiological diagnosis of bronchiectasis: what's in a name?. Eur Respir Rev 2020; 29 (156) 190120
  • 48 Costa JC, Machado JN, Ferreira C, Gama J, Rodrigues C. The Bronchiectasis Severity Index and FACED score for assessment of the severity of bronchiectasis. Pulmonology 2018; S2173-5115 (17) 30154-30159
  • 49 Kim JS, Müller NL, Park CS. et al. Bronchoarterial ratio on thin section CT: comparison between high altitude and sea level. J Comput Assist Tomogr 1997; 21 (02) 306-311
  • 50 Naidich DP, McCauley DI, Khouri NF, Stitik FP, Siegelman SS. Computed tomography of bronchiectasis. J Comput Assist Tomogr 1982; 6 (03) 437-444
  • 51 Cooke JC, Currie DC, Morgan AD. et al. Role of computed tomography in diagnosis of bronchiectasis. Thorax 1987; 42 (04) 272-277
  • 52 Lambert RK, Codd SL, Alley MR, Pack RJ. Physical determinants of bronchial mucosal folding. J Appl Physiol (1985) 1994; 77 (03) 1206-1216
  • 53 Kuo W, de Bruijne M, Petersen J. et al. Diagnosis of bronchiectasis and airway wall thickening in children with cystic fibrosis: objective airway-artery quantification. Eur Radiol 2017; 27 (11) 4680-4689
  • 54 Lynch DA, Newell JD, Tschomper BA, Cink TM, Newman LS, Bethel R. Uncomplicated asthma in adults: comparison of CT appearance of the lungs in asthmatic and healthy subjects. Radiology 1993; 188 (03) 829-833
  • 55 Milliron B, Henry TS, Veeraraghavan S, Little BP. Bronchiectasis: mechanisms and imaging clues of associated common and uncommon diseases. Radiographics 2015; 35 (04) 1011-1030
  • 56 Matsuoka S, Uchiyama K, Shima H, Ueno N, Oish S, Nojiri Y. Bronchoarterial ratio and bronchial wall thickness on high-resolution CT in asymptomatic subjects: correlation with age and smoking. AJR Am J Roentgenol 2003; 180 (02) 513-518
  • 57 Weibel ER, Gomez DM. Architecture of the human lung. Use of quantitative methods establishes fundamental relations between size and number of lung structures. Science 1962; 137 (3530): 577-585
  • 58 Kuo W, Perez-Rovira A, Tiddens H, de Bruijne M. Normal Chest CT Study Group. Airway tapering: an objective image biomarker for bronchiectasis. Eur Radiol 2020; 30 (05) 2703-2711
  • 59 Kang EY, Miller RR, Müller NL. Bronchiectasis: comparison of preoperative thin-section CT and pathologic findings in resected specimens. Radiology 1995; 195 (03) 649-654
  • 60 Kim JS, Müller NL, Park CS, Grenier P, Herold CJ. Cylindrical bronchiectasis: diagnostic findings on thin-section CT. AJR Am J Roentgenol 1997; 168 (03) 751-754
  • 61 Copley SJ, Wells AU, Hawtin KE. et al. Lung morphology in the elderly: comparative CT study of subjects over 75 years old versus those under 55 years old. Radiology 2009; 251 (02) 566-573
  • 62 Agarwal R. Bronchiectasis in acute pneumonia ... Pseudobronchiectasis. Chest 2007; 132 (06) 2054-2055
  • 63 Hughes JM. Hypoxic pulmonary vasoconstriction: clinical implications. Eur Respir J 2016; 47 (01) 31-34
  • 64 Diaz AA, Young TP, Maselli DJ. et al. Quantitative CT measures of bronchiectasis in smokers. Chest 2017; 151 (06) 1255-1262
  • 65 Jacob J, Bartholmai BJ, Rajagopalan S. et al. Mortality prediction in idiopathic pulmonary fibrosis: evaluation of computer-based CT analysis with conventional severity measures. Eur Respir J 2017; 49 (01) 1601011
  • 66 Young K, Aspestrand F, Kolbenstvedt A. High resolution CT and bronchography in the assessment of bronchiectasis. Acta Radiol 1991; 32 (06) 439-441
  • 67 Raoof S, Bondalapati P, Vydyula R. et al. Cystic lung diseases: algorithmic approach. Chest 2016; 150 (04) 945-965
  • 68 Westcott JL, Cole SR. Traction bronchiectasis in end-stage pulmonary fibrosis. Radiology 1986; 161 (03) 665-669
  • 69 Aliboni L, Pennati F, Gelmini A. et al. Detection and classification of bronchiectasis through convolutional neural networks. J Thorac Imaging 2021; (epub ahead of print March 24, 2021) DOI: 10.1097/RTI.0000000000000588.
  • 70 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 (05) 1177-1186
  • 71 Bhalla M, Turcios N, Aponte V. et al. Cystic fibrosis: scoring system with thin-section CT. Radiology 1991; 179 (03) 783-788
  • 72 Reiff DB, Wells AU, Carr DH, Cole PJ, Hansell DM. CT findings in bronchiectasis: limited value in distinguishing between idiopathic and specific types. AJR Am J Roentgenol 1995; 165 (02) 261-267
  • 73 Elborn JS. Cystic fibrosis. Lancet 2016; 388 (10059): 2519-2531
  • 74 Perry E, Eggleton P, De Soyza A, Hutchinson D, Kelly C. Increased disease activity, severity and autoantibody positivity in rheumatoid arthritis patients with co-existent bronchiectasis. Int J Rheum Dis 2017; 20 (12) 2003-2011
  • 75 Daley CL, Iaccarino JM, Lange C. et al. Treatment of nontuberculous mycobacterial pulmonary disease: an official ATS/ERS/ESCMID/IDSA Clinical Practice Guideline. Clin Infect Dis 2020; 71 (04) 905-913
  • 76 Miravitlles M, Dirksen A, Ferrarotti I. et al. European Respiratory Society statement: diagnosis and treatment of pulmonary disease in α1-antitrypsin deficiency. Eur Respir J 2017; 50 (05) 1700610
  • 77 Amati F, Franceschi E, Gramegna A, Chalmers JD, Aliberti S. Investigating the etiology of bronchiectasis: you do not find what you do not look for. Respiration 2017; 93 (03) 228-229
  • 78 Pulmonology Portuguese Society Bronchiectasis Study Group. Recommendations for aetiological diagnosis of bronchiectasis. Rev Port Pneumol (2006) 2016; 22 (04) 222-235
  • 79 Al-Jahdali H, Alshimemeri A, Mobeireek A. et al. The Saudi Thoracic Society guidelines for diagnosis and management of noncystic fibrosis bronchiectasis. Ann Thorac Med 2017; 12 (03) 135-161
  • 80 Fujita J, Ohtsuki Y, Shigeto E. et al. Pathological findings of bronchiectases caused by Mycobacterium avium intracellulare complex. Respir Med 2003; 97 (08) 933-938
  • 81 Chan ED, Iseman MD. Underlying host risk factors for nontuberculous mycobacterial lung disease. Semin Respir Crit Care Med 2013; 34 (01) 110-123
  • 82 Faverio P, Stainer A, Bonaiti G. et al. Characterizing non-tuberculous mycobacteria infection in bronchiectasis. Int J Mol Sci 2016; 17 (11) E1913
  • 83 Guan WJ, Gao YH, Xu G. et al. Aetiology of bronchiectasis in Guangzhou, southern China. Respirology 2015; 20 (05) 739-748
  • 84 McShane PJ, Naureckas ET, Strek ME. Bronchiectasis in a diverse US population: effects of ethnicity on etiology and sputum culture. Chest 2012; 142 (01) 159-167
  • 85 Lonni S, Chalmers JD, Goeminne PC. et al. Etiology of non-cystic fibrosis bronchiectasis in adults and its correlation to disease severity. Ann Am Thorac Soc 2015; 12 (12) 1764-1770
  • 86 Suarez-Cuartin G, Chalmers JD, Sibila O. Diagnostic challenges of bronchiectasis. Respir Med 2016; 116: 70-77
  • 87 Araújo D, Shteinberg M, Aliberti S. et al. Standardised classification of the aetiology of bronchiectasis using an objective algorithm. Eur Respir J 2017; 50 (06) 1701289
  • 88 Han MK, Agusti A, Calverley PM. et al. Chronic obstructive pulmonary disease phenotypes: the future of COPD. Am J Respir Crit Care Med 2010; 182 (05) 598-604
  • 89 Aliberti S, Lonni S, Dore S. et al. Clinical phenotypes in adult patients with bronchiectasis. Eur Respir J 2016; 47 (04) 1113-1122
  • 90 Chalmers JD, Aliberti S, Filonenko A. et al. Characterization of the ‘frequent exacerbator phenotype’ in bronchiectasis. Am J Respir Crit Care Med 2018; 197 (11) 1410-1420
  • 91 Martinez-Garcia MÁ, Athanazio R, Gramblicka G. et al. Prognostic value of frequent exacerbations in bronchiectasis: the relationship with disease severity. Arch Bronconeumol 2019; 55 (02) 81-87
  • 92 Perea L, Cantó E, Suarez-Cuartin G. et al. A cluster analysis of bronchiectasis patients based on the airway immune profile. Chest 2021; 159 (05) 1758-1767
  • 93 Anderson GP. Endotyping asthma: new insights into key pathogenic mechanisms in a complex, heterogeneous disease. Lancet 2008; 372 (9643): 1107-1119
  • 94 Agusti A, Bel E, Thomas M. et al. Treatable traits: toward precision medicine of chronic airway diseases. Eur Respir J 2016; 47 (02) 410-419
  • 95 Boaventura R, Sibila O, Agusti A, Chalmers JD. Treatable traits in bronchiectasis. Eur Respir J 2018; 52 (03) 1801269
  • 96 Amati F, Gramegna A, Contarini M. et al. Site of care and multidisciplinary approach. In: Chalmers JD, Polverino E, Aliberti S. eds. Bronchiectasis (ERS Monograph). Sheffield European Respiratory Society; 2018: 353-370
  • 97 Chinen J, Shearer WT. Secondary immunodeficiencies, including HIV infection. J Allergy Clin Immunol 2010; 125 (02, Suppl 2): S195-S203
  • 98 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 (01) 44
  • 99 Keir HR, Shoemark A, Dicker AJ. et al. Neutrophil extracellular traps, disease severity, and antibiotic response in bronchiectasis: an international, observational, multicohort study. Lancet Respir Med 2021; (epub ahead of print) S2213-2600(20)30504-X
  • 100 Martinez-Garcia MA, Posadas T, Sotgiu G, Blasi F, Saderi L, Aliberti S. Role of inhaled corticosteroids in reducing exacerbations in bronchiectasis patients with blood eosinophilia pooled post-hoc analysis of 2 randomized clinical trials. Respir Med 2020; 172: 106127
  • 101 Oriano M, Gramegna A, Terranova L. et al. Sputum neutrophil elastase associates with microbiota and Pseudomonas aeruginosa in bronchiectasis. Eur Respir J 2020; 56 (04) 2000769
  • 102 Aliberti S, Sotgiu G, Blasi F, Saderi L, Posadas T, Martinez Garcia MA. Blood eosinophils predict inhaled fluticasone response in bronchiectasis. Eur Respir J 2020; 56 (02) 2000453
  • 103 Bonilla FA, Khan DA, Ballas ZK. et al; Joint Task Force on Practice Parameters, representing the American Academy of Allergy, Asthma & Immunology; the American College of Allergy, Asthma & Immunology; and the Joint Council of Allergy, Asthma & Immunology. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol 2015; 136 (05) 1186-205.e1 , 78
  • 104 Gregersen S, Aaløkken TM, Mynarek G. et al. Development of pulmonary abnormalities in patients with common variable immunodeficiency: associations with clinical and immunologic factors. Ann Allergy Asthma Immunol 2010; 104 (06) 503-510
  • 105 Rodman DM, Polis JM, Heltshe SL. et al. Late diagnosis defines a unique population of long-term survivors of cystic fibrosis. Am J Respir Crit Care Med 2005; 171 (06) 621-626
  • 106 National Guideline Alliance (UK). Cystic Fibrosis: Diagnosis and management. London: National Institute for Health and Care Excellence (UK); ; October 25, 2017
  • 107 Guan WJ, Li JC, Liu F. et al. Next-generation sequencing for identifying genetic mutations in adults with bronchiectasis. J Thorac Dis 2018; 10 (05) 2618-2630
  • 108 Gramegna A, Aliberti S, Seia M. et al. When and how ruling out cystic fibrosis in adult patients with bronchiectasis. Multidiscip Respir Med 2018; 13 (Suppl. 01) 29
  • 109 Grasemann H. CFTR modulator therapy for cystic fibrosis. N Engl J Med 2017; 377 (21) 2085-2088
  • 110 Lucas JS, Barbato A, Collins SA. et al. European Respiratory Society guidelines for the diagnosis of primary ciliary dyskinesia. Eur Respir J 2017; 49 (01) 1601090
  • 111 Shoemark A, Ozerovitch L, Wilson R. Aetiology in adult patients with bronchiectasis. Respir Med 2007; 101 (06) 1163-1170
  • 112 Martínez-García MA, Soler-Cataluña JJ, Perpiñá-Tordera M, Román-Sánchez P, Soriano J. Factors associated with lung function decline in adult patients with stable non-cystic fibrosis bronchiectasis. Chest 2007; 132 (05) 1565-1572
  • 113 Contarini M, Shoemark A, Rademacher J. et al. Why, when and how to investigate primary ciliary dyskinesia in adult patients with bronchiectasis. Multidiscip Respir Med 2018; 13 (Suppl. 01) 26
  • 114 Patterson TF, Thompson III GR, Denning DW. et al. Practice guidelines for the diagnosis and management of aspergillosis: 2016 update by the Infectious Diseases Society of America. Clin Infect Dis 2016; 63 (04) e1-e60
  • 115 Li JX, Fan LC, Li MH, Cao WJ, Xu JF. Beneficial effects of Omalizumab therapy in allergic bronchopulmonary aspergillosis: a synthesis review of published literature. Respir Med 2017; 122: 33-42
  • 116 Puéchal X, Bienvenu T, Dusser D. Rheumatoid arthritis-associated bronchiectasis. Lancet 2019; 393 (10185): 2035-2036
  • 117 Desai D, Patil S, Udwadia Z, Maheshwari S, Abraham P, Joshi A. Pulmonary manifestations in inflammatory bowel disease: a prospective study. Indian J Gastroenterol 2011; 30 (05) 225-228
  • 118 Parr DG, Guest PG, Reynolds JH, Dowson LJ, Stockley RA. Prevalence and impact of bronchiectasis in alpha1-antitrypsin deficiency. Am J Respir Crit Care Med 2007; 176 (12) 1215-1221
  • 119 Patterson JE, Bradley JM, Hewitt O, Bradbury I, Elborn JS. Airway clearance in bronchiectasis: a randomized crossover trial of active cycle of breathing techniques versus Acapella. Respiration 2005; 72 (03) 239-242
  • 120 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 (05) 576-585
  • 121 Cowman S, van Ingen J, Griffith DE, Loebinger MR. Non-tuberculous mycobacterial pulmonary disease. Eur Respir J 2019; 54 (01) 1900250
  • 122 Haworth CS, Floto RA. Introducing the new BTS guideline: management of non-tuberculous mycobacterial pulmonary disease (NTM-PD). Thorax 2017; 72 (11) 969-970
  • 123 Gramegna A, Amati F, Terranova L. et al. Neutrophil elastase in bronchiectasis. Respir Res 2017; 18 (01) 211
  • 124 Aliberti S, Sotgiu G, Gramegna A. et al. Thrombocytosis during stable state predicts mortality in bronchiectasis. Ann Am Thorac Soc 2021; (epub ahead of print January 28, 2021) DOI: 10.1513/AnnalsATS.202002-094OC.
  • 125 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 (01) 1702557
  • 126 Mandal P, Morice AH, Chalmers JD, Hill AT. Symptoms of airway reflux predict exacerbations and quality of life in bronchiectasis. Respir Med 2013; 107 (07) 1008-1013
  • 127 Chalmers JD, McHugh BJ, Docherty C, Govan JR, Hill AT. Vitamin-D deficiency is associated with chronic bacterial colonisation and disease severity in bronchiectasis. Thorax 2013; 68 (01) 39-47
  • 128 Mathyssen C, Gayan-Ramirez G, Bouillon R, Janssens W. Vitamin D supplementation in respiratory diseases: evidence from randomized controlled trials. Pol Arch Intern Med 2017; 127 (11) 775-784