Update on the management of patients with non cystic fibrosis bronchiectasis
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5th Pulmonary Clinic, Sotiria Chest Diseases Hospital, Athens, Greece
6th Pulmonary Clinic, Sotiria Chest Diseases Hospital, Athens, Greece
Corresponding author
Katerina Dimakou   

152 Mesogion Ave., 11527 Athens, Greece
Pneumon 2015;28(3):251-257
Bronchiectasis not due to cystic fibrosis (non CF bronchiectasis) is an underdiagnosed disease, while recently an increase in the prevalence and hospitalizations for bronchiectasis has been reported, causing a substantial burden on healthcare systems. The goal of the appropriate management of patients with non CF bronchiectasis is to cease the “vicious circle” of bronchiectasis. After an initial thorough aetiological investigation, general measures should be offered that include smoking cessation, vaccination against influenza and pneumonococcal infection and oxygen supplementation if respiratory failure occurs. Antibiotics are the cornerstone of treatment. The appropriate antibiotic regimen guided by sputum culture is of great importance in exacerbations of bronchiectasis, while certain algoriths exist for the eradication of Pseudomonas aeruginosa. Long term macrolides and inhaled antibiotics are recommended for patients chronically colonized with P.aeruginosa and in patients who present ≥3 exacerbations per year or even in case of fewer exacerbations but with significant morbidity, as they have shown positive effects in sputum volume and purulence, they reduce the risk of exacerbations and improve symptoms and quality of life. A subset of patients with a significant bronchodilator response and obstructive lung function pattern may benefit from inhaled β2-agonists and/or inhaled corticosteroids. Airway clearance techniques and pulmonary rehabilitation have been found to improve sputum expectoration, reduce hyperinflation and quality of life. Bronchial arterial embolization is the treatment of choice in cases of severe or reccurent haemoptysis, while surgery for bronchiectasis is extremely rare nowadays. New therapies are currently under consideration, while updated recommendations and guidelines are under preparation.
The authors have indicated no financial conflicts of interest.
Barker AF. Bronchiectasis. N Engl J Med 2002; 346:1383-93.
Pasteur MC, Helliwell SM, Houghton SJ, et al. An investigation into causative factors in patients with bronchiectasis. Am J Respir Crit Care Med 2000; 162:1277-84.
Pasteur MC, Bilton D, Hill AT. British Thoracic Society guideline for non-CF bronchiectasis. Thorax 2010; 65: Suppl. 1:i1–i58.
McGuinness G, Naidich DP, Leitman BS, et al. Bronchiectasis: CT evaluation. Am J Roentgenol 1993; 160:253-9.
McGuinness G, Naidich DP. CT of airways disease and bronchiectasis. Radiol Clin North Am 2002; 40:1–19.
Chalmers JD, Aliberti S, Blasi F. Management of bronchiectasis in adults. Eur Respir J 2015; 45:1446-62.
Anwar GA, McDonnell MJ, Worthy SA, et al. Phenotyping adults with non-cystic fibrosis bronchiectasis: aprospective observational cohort study. Respir Med 2013; 107:1001-7.
Dimakou K, Triantafillidou C, Toumbis M, Tsikritsaki K, Malagari K, Bakakos P. Non CF-bronchiectasis: Aetiologic approach, clinical, radiological, microbiological and functional profile in a Greek population. Submitted.
Vendrell M, de Gracia J, Olveira C, et al. Diagnosis and treatment of bronchiectasis. Spanish Society of Pneumology and Thoracic Surgery.Arch Bronconeumol 2008; 44:629-40.
Shoemark A, Ozerovitch L, Wilson R. Aetiology in adult patients with bronchiectasis. Respir Med 2007; 101:1163-70.
Chalmers JD, Goeminne P, Aliberti S. The Bronchiectasis Severity Index. An International Derivation and Validation Study. Am J Res Crit Care Med 2014; 189:576-85.
Matera MG. Pharmacologic characteristics of prulifloxacin. Pulm Pharmacol Ther 2006;19 Suppl 1:20-9.
Blasi F, Schaberg T, Centanni S. Prulifloxacin versus levofloxacin in the treatment of severe COPD patients with acute exacerbations of chronic bronchitis. Pulm Pharmacol Ther 2013; 26:609-16.
Onen ZP, Gulbay BE, SenE, et al. Analysis of the factors related to mortality in patients with bronchiectasis. Respir Med 2007; 101:1390-7.
Loebinger MR, Wells AU, Hansell DM. Mortality in bronchiectasis: a long-term study assessing the factors influencing survival. Eur Respir J 2009; 34:843-9.
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; 309:1251-9.
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; 380:660-7.
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; 2:988-96.
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; 309:1260-7.
E. Yalcin, N. Kiper, U. Ozcelik, et al. Effects of clarithromycin on inflammatory parameters and clinical conditions in children with bronchiectasis. J Clin Pharm Ther 2006; 31:49-55.
Quon BS, Goss CH, Ramsey BW. Inhaled antibiotics for lower airway infections.Ann Am ThoracSoc 2014; 1:425-34.
Brodt AM, Stovold E, Zhang L. Inhaled antibiotics for stable non-cystic fibrosis bronchiectasis: a systematic review. Eur Respir J 2014; 44:382-93.
Rosales-Mayor E, Serrano VA, Polverino E. Inhaled antibiotics in bronchiectasis. Community Acquir Infect 2015;2:8-12.
Franco F, Sheikh A, Greenstone M. Short acting beta-2 agonists for bronchiectasis.Cochrane Database Syst Rev 2003(3):CD003572.
Lasserson T, Holt K, Evans D, et al. Anticholinergic therapy for bronchiectasis. Cochrane Database Syst Rev 2001(4):CD002163.
Abu Hassan J, Saadiah S, Roslan H, et al. Bronchodilator response to inhaled beta-2 agonist and anticholinergic drugs in patients with bronchiectasis. Respirology 1999; 4:423-6.
Steele K, Greenstone M, Lasserson JA. Oral methyl-xanthines for bronchiectasis. Cochrane Database Syst Rev 2001(1):CD002734.
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; 100:1623-32.
Martinez-Garcia MA, Soler-Cataluna JJ, Catalan-Serra P, et al. Clinical efficacy and safety of budesonide-formoterol in noncystic fibrosis bronchiectasis. Chest 2012; 141:461-8.
Singanayagam A, Chalmers JD, AkramAR, et al. Impact of inhaled corticosteroid use on outcome in COPD patients admitted with pneumonia. Eur Respir J 2011; 38:36-41.
Lee AL, Burge A, Holland AE. Airway clearance techniques for bronchiectasis. Cochrane Database Syst Rev 2013; 5: CD008351.
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.
Bilton D, Daviskas E, AndersonSD, 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; 144:215-25.
Bilton D, Tino G, Barker AF, et al. Inhaled mannitol for non-cystic fibrosis bronchiectasis: a randomised, controlled trial. Thorax 2014; 69: 1073-9.
Olivieri D, Ciaccia A, Marangio E, et al. Role of bromhexine in exacerbations of bronchiectasis. Double-blind randomized multicenter study versus placebo. Respiration 1991; 58:117-21.
O’Donnell AE, Barker AF, Ilowite JS, et al. Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group. Chest 1998; 113:1329-34.
Mandal P, Sidhu MK, Kope L, et al. A pilot study of pulmonary rehabilitation and chest physiotherapy versuschest physiotherapy alone in bronchiectasis. Respir Med 2012; 106:1647-54.
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.
Meléndez Torres JR, Padua y Gabriel A, Velasco Rodriguez VM, et al. Survival after bronchial artery embolization in massive hemoptysis: experience in 24 cases. J Bronchol 2003; 10:17-21.
Sharma S, Kothari SS, Bhargava AD, et al. Transcatheterindigeneous coil embolization in recurrent massive hemoptysis secondary to post-tubercular bronchiectasis. J Assoc Physicians India 1995; 43:127-9.
Wong ML, Szkup P, Hopley MJ. Percutaneous embolotherapy for life-threatening hemoptysis. Chest 2002;121:95-102.
Phase 2, Single Group, Open Clinical Trial to Evaluate the Efficacy and Safety of Roflumilast in Symptomatic Bronchiectasis Patients. identifier: NCT01580748.
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; 107:524-33.
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