Management of bronchiectasis in adults

European Respiratory Journal

Volumn 45, Issue 5, 2015, Pages 1446-1462

  Chalmers, James D ^a   Aliberti, Stefano ^b   Blasi, Francesco ^c  

^a UNIVERSITY OF DUNDEE   (United Kingdom)

^b UNIVERSITY OF MILANO BICOCCA   (Italy)

^c UNIVERSITY OF MILAN   (Italy)

Author keywords

[No Author keywords available]

Indexed keywords

ACETYLCYSTEINE; AMOXICILLIN; AMOXICILLIN PLUS CLAVULANIC ACID; ATORVASTATIN; AZITHROMYCIN; AZTREONAM; BETA ADRENERGIC RECEPTOR STIMULATING AGENT; BUDESONIDE; BUDESONIDE PLUS FORMOTEROL; CARBOCISTEINE; CIPROFLOXACIN; COLISTIN; CORTICOSTEROID; DEOXYRIBONUCLEASE; ERYTHROMYCIN ETHYLSUCCINATE; GENTAMICIN; LEUKOCYTE ELASTASE INHIBITOR; MACROLIDE; MANNITOL; N [2 (2,3 DIFLUOROBENZYLTHIO) 6 (2,3 DIHYDROXY 1 METHYLPROPOXY) 4 PYRIMIDINYL] 1 AZETIDINESULFONAMIDE; PLACEBO; SODIUM CHLORIDE; TETRACYCLINE DERIVATIVE; TOBRAMYCIN; ANTIINFECTIVE AGENT; ANTIINFLAMMATORY AGENT; BRONCHODILATING AGENT;

ADULT; ANTIBIOTIC RESISTANCE; ARTICLE; BREATHING EXERCISE; BRONCHIECTASIS; BRONCHOCONSTRICTION; BRONCHOSPASM; COMORBIDITY; COUGHING; DISEASE ERADICATION; DISEASE EXACERBATION; DISEASE SEVERITY; DRUG DOSE REDUCTION; DRUG FORMULATION; DRUG MEGADOSE; DRUG SAFETY; DRUG WITHDRAWAL; DYSPNEA; GASTROINTESTINAL SYMPTOM; HEALTH CARE MANAGEMENT; HUMAN; LUNG CLEARANCE; LUNG LOBECTOMY; NEBULIZER; NEPHROTOXICITY; OTOTOXICITY; PHASE 2 CLINICAL TRIAL (TOPIC); PHASE 3 CLINICAL TRIAL (TOPIC); PNEUMONIA; POSITIVE END EXPIRATORY PRESSURE; POSITIVE END EXPIRATORY PRESSURE VENTILATOR; PRACTICE GUIDELINE; PRIORITY JOURNAL; PULMONARY REHABILITATION; RANDOMIZED CONTROLLED TRIAL (TOPIC); SYSTEMATIC REVIEW; THORAX PAIN; TREATMENT RESPONSE; UNSPECIFIED SIDE EFFECT; WHEEZING; BACTERIAL INFECTIONS; CHRONIC OBSTRUCTIVE LUNG DISEASE; COHORT ANALYSIS; CYSTIC FIBROSIS; INFLAMMATION; ORAL DRUG ADMINISTRATION; PATHOPHYSIOLOGY; PREVALENCE; PROCEDURES; PROGNOSIS; PSYCHOLOGY; PULMONOLOGY; QUALITY OF LIFE; TRENDS;

ADMINISTRATION, ORAL; ADRENAL CORTEX HORMONES; ADULT; ANTI-BACTERIAL AGENTS; ANTI-INFLAMMATORY AGENTS; BACTERIAL INFECTIONS; BRONCHIECTASIS; BRONCHODILATOR AGENTS; COHORT STUDIES; COMORBIDITY; CYSTIC FIBROSIS; HUMANS; INFLAMMATION; PREVALENCE; PROGNOSIS; PULMONARY DISEASE, CHRONIC OBSTRUCTIVE; PULMONARY MEDICINE; QUALITY OF LIFE; RANDOMIZED CONTROLLED TRIALS AS TOPIC;

EID: 84929017972     PISSN: 09031936     EISSN: 13993003     Source Type: Journal    
DOI: 10.1183/09031936.00119114     Document Type: Article

References (116)

1. Chalmers JD, Hill AT. Mechanisms of immune dysfunction and bacterial persistence in non-cystic fibrosis bronchiectasis. Mol Immunol 2013; 55: 27-34.
2. Pasteur MC, Bilton D, Hill AT. British Thoracic Society guideline for non-CF bronchiectasis. Thorax 2010; 65: Suppl. 1, i1-i58.
3. Vendrell M, De Gracia J, Olveira C, et al. Diagnosis and treatment of bronchiectasis. Spanish Society of Pneumology and Thoracic Surgery. Arch Broncopneumol 2008; 44: 629-640.
4. Ringshausen FC, de Roux A, 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.
5. Seitz AE, Olivier KN, Adjemian J, et al. Trends in bronchiectasis among Medicare beneficiaries in the United States, 2000 to 2007. Chest 2012; 142: 432-439.
6. De Soyza A, Brown JS, Loebinger MR. Research priorities in bronchiectasis. Thorax 2013; 68: 695-696.
7. Chalmers JD, Goeminne P, Aliberti S, et al. Derivation and validation of the bronchiectasis severity index: An international multicentre observational study. Am J Respir Crit Care Med 2014; 189: 576-585.
8. Chalmers JD, McHugh BJ, Doherty CJ, et al. Vitamin-D deficiency is associated with chronic bacterial colonisation and disease severity in non-CF bronchiectasis. Thorax 2012; 68: 39-47.
9. 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.
10. Goeminne PC, Nawrot TS, Ruttens D, et al. Mortality in non-cystic fibrosis bronchiectasis: A prospective cohort analysis. Respir Med 2014; 108: 287-296.
11. Fuschillo S, De Felice A, Balzano G. Mucosal inflammation in idiopathic bronchiectasis: cellular and molecular mechanisms. Eur Respir J 2008; 31: 396-406.
12. Tsang KW, Chan KN, Ho P, et al. Sputum elastase in steady-state bronchiectasis. Chest 2000; 117: 420-426.
13. Mikami M, Llewellyn-Jones CG, Bayley D, et al. The chemotactic activity of sputum from patients with bronchiectasis. Am J Respir Crit Care Med 1998; 157: 723-728.
14. Cole PJ. Inflammation: A two-edged sword-the model of bronchiectasis. Eur J Respir Dis Suppl 1986; 147: 6-15.
15. King PT, Hutchinson P, Holmes PW, et al. Assessing immune function in adult bronchiectasis. Clin ExpImmunol 2006; 144: 440-446.
16. Ruchaud-Sparagnano MH, Gertig H, Hester KL, et al. Effect of granulocyte-macrophage colony-stimulating factor on neutrophil function in idiopathic bronchiectasis. Respirology 2013; 18: 1230-1235.
17. Voynow JA, Young LR, Wang Y, et al. Neutrophil elastase increases MUC5AC mRNA and protein expression in respiratory epithelial cells. Am J Physiol 1999; 276: L835-L843.
18. Amitani R, Wilson R, Rutman A, et al. Effects of human neutrophil elastase and Pseudomonas aeruginosa proteinases on human respiratory epithelium. Am J Respir Cell Mol Biol 1991; 4: 26-32.
19. Tosi MF, Zakem H, Berger M. Neutrophil elastase cleaves C3bi on opsonized pseudomonas as well as CR1 on neutrophils to create a functionally important opsonin receptor mismatch. J Clin Invest 1990; 86: 300-308.
20. Berger M, Sorensen RU, Tosi MF, et al. Complement receptor expression on neutrophils at an inflammatory site, the pseudomonas infection lung in cystic fibrosis. J Clin Invest 1989; 84: 1302-1313.
21. Voglis S, Quinn K, Tullis E, et al. Human neutrophil peptides and phagocytic deficiency in bronchiectatic lungs. Am J Respir Crit Care Med 2009; 180: 159-166.
22. Vandivier RW, Fadok VA, Hoffman PR. Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis. J Clin Invest 2002; 109: 661-670.
23. Tan HL, Regamey N, Brown S, et al. The Th17 pathway in cystic fibrosis lung disease. Am J Respir Crit Care Med 2011; 184: 252-258.
24. Marsland BJ, Yadava K, Nicod LP. The airway microbiome and disease. Chest 2013; 144: 632-637.
25. Tunney MM, Einarsson GG, Wei L, et al. Lung microbiota and bacterial abundance in patients with bronchiectasis when clinically stable and during exacerbation. Am J Respir Crit Care Med 2013; 187: 1118-1126.
26. Rogers GB, van der Gast CJ, Serisier DJ. Predominant pathogen comptetition and core microbiota divergence in chronic airway infection. ISME J 2014; 9: 217-225.
27. Rogers GB, van der Gast CJ, Cuthbertson L, et al. Clinical measures of disease in adult non-CF bronchiectasis correlate with airway microbiota composition. Thorax 2013; 68: 731-737.
28. Rogers GB, Zain NM, Bruce KD, et al. A novel microbiota stratification system predicts future exacerbations in bronchiectasis. Ann Am Thorac Soc 2014; 11: 496-503.
29. Wells TJ, Whitters D, Sevastsyanovich YR, et al. Increased severity of respiratory infections associated with elevated anti-LPS IgG2 wich inhibits serum bactericidal killing. J Exp Med 2014; 211: 1893-1904.
30. Chalmers JD, Fleming GB, Hill AT, Kilpatrick DC. Impact of mannose-binding lectin insufficiency on the course of cystic fibrosis: A review and meta-analysis. Glycobiology 2011; 21: 271-282.
31. Fevang B, Mollnes TE, Holm AM, et al. Common variable immunodeficiency and the complement system; low mannose binding lectin levels are associated with bronchiectasis. Clin Exp Immunol 2005; 142: 576-584.
32. Pifferi M, Bush A, Michelucci A, et al. Mannose binding lectin 2 gene polymorphisms and lung damage in primary ciliary dyskinesia. Pediatr Pulmonol 2015; 50: 179-186.
33. Chalmers JD, McHugh BJ, Doherty C, et al. Mannose binding lectin deficiency and disease severity in non-cystic fibrosis bronchiectasis: A prospective study. Lancet Respir Med 2013; 1: 224-232.
34. Shoemark A, Ozerovitch L, Wilson R. Aetiology in adult patients with bronchiectasis. Respir Med 2007; 101: 1163-1170.
35. 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.
36. 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.
37. Kelly MG, Murphy S, Elborn JS. Bronchiectasis in secondary care: A comprehensive profile of a neglected disease. Eur J Intern Med 2003; 14: 488-492.
38. Winthrop KL, Aksamit TR, Olivier KN, et al. The respiratory microbiology of patients with nontuberculous mycobacteria from the United States Bronchiectasis Research Registry. Am J Respir Crit Care Med 2013; 187: A4541.
39. McShane PJ, Naureckas ET, Strek ME. Bronchiectasis in a diverse US population: effects of ethnicity on etiology and sputum culture. Chest 2012; 142: 159-167.
40. Agusti A, Calverley PM, Celli B, et al. Characterisation of COPD heterogeneity in the ECLIPSE cohort. Respir Res 2010; 11: 122.
41. Martinez-Garcia MA, Soler-Cataluna JJ, Donat Sanz Y, et al. Factors associated with bronchiectasis in patients with COPD. Chest 2011; 140: 1130-1137.
42. Paganin F, Seneterre E, Chanez P, et al. Computed tomography of the lungs in asthma: influence of disease severity and etiology. Am J Respir Crit Care Med 1996; 153: 110-114.
43. Mandal P, Morice A, Chalmers JD, Hill AT. Symptoms of airway reflux predict exacerbations and quality of life in bronchiectasis. Respir Med 2013; 107: 1008-1013.
44. Flude LJ, Agent P, Bilton D. Chest physiotherapy techniques in bronchiectasis. Clin Chest Med 2012; 33: 351-361.
45. Bott J, Blumenthal S, Buxton M, et al. Guidelines for the physiotherapy management of the adult medical spontaneously breathing patient. Thorax 2009; 64: Suppl. 1, i1-i51.
46. Murray MP, Pentland JL, Hill AT. A randomised crossover trial of chest physiotherapy in non-cystic fibrosis bronchiectasis. Eur Respir J 2009; 34: 1086-1092.
47. Lee AL, Burge A, Holland AE. Airway clearance techniques for bronchiectasis. Cochrane Database Syst Rev 2013; 5: CD008351.
48. Ong HK, Lee AL, Hill CJ, et al. Effects of pulmonary rehabilitation in bronchiectasis: A retrospective study. Chron Respir Dis 2011; 8: 21-30.
49. Mandal P, Sidhu MK, Kope L, et al. A pilot study of pulmonary rehabilitation and chest physiotherapy versus chest physiotherapy alone in bronchiectasis. Respir Med 2012; 106: 1647-1654.
50. 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.
51. Kellet F, Robert NM. Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis. Respir Med 2011; 105: 1831-1835.
52. 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.
53. O'Donnell 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.
54. Hill AT, Welham S, Reid K, et al. British Thoracic Society national bronchiectasis audit 2010 and 2011. Thorax 2012; 67: 928-930.
55. 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.
56. Bilton D, Tino G, Barker AF, et al. Inhaled mannitol for non-cystic fibrosis bronchiectasis: A randomised, controlled trial. Thorax 2014; 69: 1073-1079.
57. 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: 1251-1259.
58. 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: 1260-1267.
59. 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.
60. Rogers GB, Brice KD, Martin ML, et al. The effect of long-term macrolide treatment on respiratory microbiota composition in non-cystic fibrosis bronchiectasis: An analysis from the randomised double blind placebo controlled BLESS trial. Lancet Respir Med 2014; 2: 988-996.
61. Ray WA, Murray KT, Hall K, et al. Azithromycin and the risk of cardiovascular death. N Engl J Med 2012; 366: 1881-1890.
62. Albert RK, Connett J, Bailey WC, et al. Azithromycin for prevention of exacerbations of COPD. N Engl J Med 2011; 365: 689-698.
63. Altenburg J, de Graaff CS, Van der Werf TS, et al. Immunomodulatory effects of macrolide antibiotics. Respiration 2011; 81: 75-87.
64. Levert H, Gressier B, Moutard I, et al. Azithromycin impact on neutrophil oxidative metabolism depends on exposure time. Inflammation 1998; 22: 191-201.
65. Elborn JS, Tunney MM. Macrolides and bronchiectasis: clinical benefit with a resistance price. JAMA 2013; 309: 1295-1296.
66. Crooks MG, Hart SP, Morice AH. Azithromyci for prevention of exacerbations of COPD. N Engl J Med 2011; 365: 2234-2235.
67. Wu Q, Shen W, Cheng H, Zhou X. Long-term macrolides for non-cystic fibrosis bronchiectasis: A systematic review and meta-analysis. Respirology 2014; 19: 321-329.
68. Yang IA, Clarke MS, Sim EH, et al. Inhaled corticosteroids for stable chronic obstructive pulmonary disease. Cochrane Database Syst Rev 2012; 7: CD002991.
69. 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.
70. 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.
71. Goyal V, Chang AB. Combined inhaled corticosteroids and long acting beta2-agonists for children and adults with bronchiectasis. Cochrane Database Syst Rev 2014; 6: CD010327.
72. Kapur N, Bell S, Kolbe J, Chang AB. Inhaled steroids for bronchiectasis. Cochrane Database Syst Rev 2009; CD000996.
73. Singanayagam A, Chalmers JD, Akram AR, et al. Impact of inhaled corticosteroid use on outcome in COPD patients admitted with pneumonia. Eur Respir J 2011; 38: 36-41.
74. 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.
75. Quon BS, Goss CH, Ramsey BW. Inhaled antibiotics for lower airway infections. Ann Am Thorac Soc 2014; 11: 425-434.
76. Chalmers JD, Smith MP, McHugh B, et al. Short and long term antibiotic therapy reduces airway and systemic inflammation in non-CF bronchiectasis. Am J Respir Crit Care Med 2012; 186: 657-665.
77. Littlewood KJ, Higashi K, Jansen JP, et al. A network meta-analysis of the efficacy of inhaled antibiotics for chronic Pseudomonas infections in cystic fibrosis. J Cyst Fibros 2012; 11: 419-426.
78. Stockley RA, Hill SL, Burnett D. Nebulized amoxicillin in chronic purulent bronchiectasis. Clin Ther 1985; 7: 593-599.
79. Hill SL, Burnett D, Hewetson KA, et al. The response of patients with purulent bronchiectasis to antibiotics for four months. Q J Med 1988; 66: 163-173.
80. Hill SL, Morrison HM, Burnett D, et al. Short term response of patients with bronchiectasis to treatment with amoxycillin given in standard or high doses orally or by inhalation. Thorax 1986; 41: 559-565.
81. 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.
82. Murray MP, Govan JRW, Docherty CJ, et al. A randomised controlled trial of nebulised gentamicin in non-cystic fibrosis bronchiectasis. Am J Respir Crit Care Med 2011; 183: 491-499.
83. 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.
84. Barker AF, O'Donnell 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.
85. Quittner AL, O'Donnell AE, Salathe MA, et al. Quality of life questionnaire-Bronchiectasis: final psychometric analyses and determination of minimal important difference scores. Thorax 2014; 70: 12-20.
86. Wilson R, Welte T, Polverino E, et al. Ciprofloxacin dry powder for inhalation in non-cystic fibrosiss bronchiectasis: A phase II randomised study. Eur Respir J 2013; 41: 1108-1115.
87. 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.
88. McCullough AR, Tunney M, Elborn JS, et al. All illness is personal to that individual': A qualitative study of patients perspective on treatment adherence in bronchiectasis. Health Expect 2014; [In press DOI:10.1111/hex.12217].
89. Martinez-Garcia MA, de Gracia J, Vendrell Relat M et al. Multidomensional approach to non-cystic fibrosis bronchiectasis; the FACED score. Eur Respir J 2014; 43: 1357-1367.
90. 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.
91. McDonnell MK, Jary HR, Perry A, et al. Non cystic fibrosis bronchiectasis: A longitudinal retrospective observational cohort study of Pseudomonas persistence and resistance. Respir Med 2014; [In press DOI:10.1016/j.rmed.2014.07.021].
92. Murray MP, Turnbull K, Macquarrie S, Hill AT. Assessing response to treatment of exacerbations of bronchiectasis in adults. Eur Respir J 2009; 33: 312-318.
93. Zhang P, Zhang F, Jiang S, et al. Videoassisted thoracic surgery for bronchiectasis. Ann Thorac Surg 2011; 91: 239-243.
94. Vallilo CC, Terra RM, de Albuquerque AL et al. Lung resection improves the quality of life of patients with symptomatic bronchiectasis. Ann Thorac Surg 2014; 3: 1034-1041.
95. Boussoffara L, Boudawara N, Gharsallaoui Z, et al. Anxiety-depressive disorders and bronchiectasis. Rev Mal Respir 2014; 31: 230-236.
96. Giron Moreno RM, Fernandes Vasconcelos G, Cisneros C, et al. Presence of anxiety and depression in patients with bronchiectasis unrelated to cystic fibrosis. Arch Bronconeumol 2013; 49: 415-420.
97. O'Leary CJ, Wilson CB, Hansell DM, et al. Relationship between psychological well-being and lung health status in patients with bronchiectasis. Respir Med 2002; 96: 686-692.
98. Poppelwell L, Chalmers JD. Defining severity in non-cystic fibrosis bronchiectasis. Expert Rev Respir Med 2014; 8: 249-262.
99. Martinez-Garcia MA, Soler-Cataluna JJ, Perpina-Tordera M, et al. Factors associated with lung function decline in adult patients with stable non-cystic fibrosis bronchiectasis. Chest 2007; 132: 1565-1572.
100. Serisier DJ. Risk of population antimicrobial resistance associated with chronic macrolide use for inflammatory airway diseases. Lancet Respir Med 2013; 1: 262-274.
101. Vestbo J, Hurd SS, Agusti AG, et al. Global strategy for the diagnosis, management, and prevention of chronic obstructive pulmonary disease: GOLD executive summary. Am J Respir Crit Care Med 2013; 187: 347-365.
102. Levy ML, Thomas M, Small I, et al. Summary of the 2008 BTS/SIGN British Guideline on the management of asthma. Prim Care Respir J 2009; 18: Suppl. 1, S1-S16.
103. Barnes PJ. Corticosteroid resistance in patients with asthma and chronic obstructive pulmonary disease. J Allergy Clin Immunol 2013; 131: 636-645.
104. Chan SC, Shum DK, Ip MS. Sputum sol neutrophil elastase activity in bronchiectasis: differential modulation by syndecan-1. Am J Respir Crit Care Med 2003; 168: 192-198.
105. 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.
106. Wu Y, Wang S, Farooq SM, et al. A chemokine receptor CXCR2 macromolecular complex regulates neutrophil functions in inflammatory diseases. J Biol Chem 2012; 287: 5744-5755.
107. Chapman RW, Philips JE, Hipkin RW, et al. CXCR2 antagonists for the treatment of pulmonary disease. Pharmacol Ther 2009; 121: 55-68.
108. Pavord I, De Soyza A, Elborn JS, et al. Efficacy and safety of AZD5069, a CXCR2 antagonist in adult bronchiectasis. Eur Respir J 2013; 42: Suppl. 57, P1593.
109. Doring G, Bragonzi A, Paroni M, et al. BIL 284 reduces neutrophil numbers but increases P. aeruginosa bacteremia and inflammation in mouse lungs. J Cyst Fibros 2014; 13: 156-163.
110. Konstan MW, Doring G, Heltsche SL, et al. A randomized double blind, placebo controlled phase 2 trial of BIIL284BS (an LTB4 receptor antagonist) for the treatment of lung disease in children and adults with cystic fibrosis. J Cyst Fibros 2014; 13: 148-155.
111. 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.
112. Srinivas N, Jetter P, Ueberbacher BJ, et al. Peptidomimetic antibiotics target outer-membrane biogenesis in Pseudomonas aeruginosa. Science 2010; 327: 1010-1013.
113. Milla CE, Chmiel JF, Accurso FJ, et al. Anti-PcrV antibody in cystic fibrosis: A novel approach targeting Pseudomonas aeruginosa airway infection. Pediatr Pulmonol 2014; 49: 650-658.
114. Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 2011; 365: 1663-1672.
115. McCormick J, Mehta G, Olesen HV, et al. Comparative demographics of the European cystic fibrosis population: A cross-sectional database analysis. Lancet 2010; 375: 1007-1013.
116. Aksamit TR, Carretta E, Daley CL, et al. The Bronchiectasis Research Registry: A Collaborative Research Cohort for Non-Cystic Fibrosis Bronchiectasis. Am J Respir Crit Care Med 2012; A3654.