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Bronchiectasis

Last updated: August 24, 2021

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Bronchiectasis is an irreversible and abnormal dilation in the bronchial tree caused by cycles of bronchial inflammation leading to mucous plugging and progressive airway destruction. Bronchiectasis is classified according to etiology as either cystic fibrosis (CF) bronchiectasis or non-CF bronchiectasis (e.g., secondary to severe or protracted pneumonia, immunodeficiency, or COPD). The characteristic clinical feature is a chronic cough with copious mucopurulent sputum. Other symptoms may include dyspnea, rhinosinusitis, and hemoptysis. Physical examination reveals crackles and rhonchi on auscultation, often accompanied by wheezing. High-resolution computed tomography confirms the diagnosis and usually shows thickened bronchial walls, with so-called “signet-ring” and “tram track” signs. Patients can periodically experience an acute worsening of symptoms, referred to as an acute exacerbation of bronchiectasis, which commonly requires antibiotic treatment. The goal of long-term management of bronchiectasis is to control symptoms and prevent exacerbations, and includes pulmonary physiotherapy and pharmacological therapy. Massive hemoptysis is a rare complication of bronchiectasis and requires surgery or pulmonary artery embolization.

  • Bronchiectasis: an irreversible and abnormal dilation of the bronchial tree that produces chronic respiratory symptoms (e.g., chronic productive cough)
  • Acute exacerbation of bronchiectasis: a deterioration in the symptoms of bronchiectasis that requires a change in the regular treatment (e.g., adding antibiotics, increasing airway clearance techniques) [1]

Bronchiectasis requires the combination of two important processes taking place in the bronchi: either local infection or inflammation along with either inadequate clearance of secretions, airway obstruction, or impaired host defenses. These processes result in the permanent dilation of airways.

Primary prevention of bronchiectasis includes antibiotic control of bronchial and pulmonary infections in predisposed individuals.

References:[2][3][4]

Bronchiectasis should be suspected in patients with a chronic cough that produces large amounts of sputum.
References: [4][6]

Approach [7]

  • Confirm the diagnosis with imaging studies in patients with features suggestive of bronchiectasis.
    • Poor control or frequent exacerbations in patients with chronic pulmonary disease (e.g., COPD, asthma)
    • Chronic cough or recurrent pulmonary infections in patients with immunosuppression or conditions associated with bronchiectasis
    • Productive cough lasting > 8 weeks in otherwise healthy patients
  • Identify the underlying etiology.

In patients with suspected bronchiectasis, diagnosis is confirmed using imaging studies, preferably a HRCT scan. Additional diagnostic studies are useful to identify the underlying cause and possibly provide specific treatment.

Imaging [7][8][9]

Identification of the underlying etiology

Studies to determine the underlying etiology of bronchiectasis are usually requested by a specialist. In many cases, it is not possible to reach a specific diagnosis and the disease may be classified as idiopathic. [10]

Initial workup [7][11]

Perform at the time of diagnosis to obtain baseline references. Sputum culture and smear, as well as spirometry, are also used for monitoring.

Additional diagnostics [7][11]

These studies may be considered depending on clinical suspicion if the initial workup was not diagnostic.

If the initial workup does not identify the underlying cause, perform further studies guided by the patient's clinical features and consider referral to a specialist.

This section focuses on the management of non-CF bronchiectasis. See “Cystic fibrosis” for specific recommendations regarding that condition.

Approach [7][11][14]

Monitoring and disposition [7][11]

Common empiric antibiotic regimens for bronchiectasis exacerbations [7][11]
Outpatient treatment [7][14]
Inpatient treatment [7][14]

Acute exacerbations are defined as acute deterioration or worsening local symptoms and/or additional systemic symptoms such as fever or malaise. Exacerbations are associated with increased inflammation and progressive damage to the lungs. [11]

Management goals are to stop or delay disease progression, reduce exacerbation frequency (goal ≤ 2 per year), achieve symptom control, and improve the patient's quality of life.

Approach [7][11]

Perform a careful reassessment of patients who are progressively deteriorating (i.e., patients with increased frequency and/or severity of exacerbations, frequent hospital admissions, worsening symptoms, rapid decline in lung function). Identify the cause of bronchiectasis if still unknown, and exclude any comorbidities or exacerbating conditions such as new pathogen colonization.

For patients with bronchiectasis and chronic productive cough or difficulty expectorating, consider referral to a trained respiratory physiotherapist for airway clearance techniques. [15]

Pharmacotherapy for airway clearance in bronchiectasis

Mucoactive agents

Consider in patients with difficulty expectorating or persistent symptoms despite adequate airway clearance techniques; continue use based on clinical benefits. There is a paucity of evidence for the benefit of mucoactive agents in bronchiectasis. [7][16]

Bronchodilators

Consider on a case-by-case basis. Evidence to support the use of bronchodilators in bronchiectasis is limited. [7][11]

Corticosteroids

Corticosteroids are not routinely recommended because of the limited benefit and side effects of corticosteroid therapy. [7][20]

For patients receiving multiple inhaled treatments and chest physiotherapy, consider the following order of treatment to avoid bronchospasm: bronchodilators, mucoactive agents, respiratory physiotherapy, inhaled antibiotics. [11]

Avoid treatment with recombinant human DNase (e.g., dornase alfa), as it can increase the frequency of exacerbations in patients with non-CF bronchiectasis. [7][21]

Long-term antibiotic therapy [7][11][14]

The goal is to suppress bacterial growth and to reduce symptoms and exacerbations as a measure of secondary prevention in patients with ≥ 3 exacerbations per year. Antibiotic therapy should be administered for at least 3 months and may be extended based on clinical response and tolerability.

Before starting treatment, obtain new sputum cultures with an antibiogram and consult an infectious diseases specialist.

Long-term antibiotic therapy for bronchiectasis
No colonization with P. aeruginosa
Colonization with P. aeruginosa [7][11]
Isolation of other pathogens

Chronic macrolide use can increase the growth of macrolide-resistant mycobacterial strains. It is recommended to rule out active nontuberculous mycobacterial infection with a negative culture before starting treatment.

Long-term macrolides can lead to QT-prolongation and fatal arrhythmias. Before starting treatment, perform an ECG and review whether any medications the patient is taking are known to alter the QT interval. [7][10]

Invasive procedures [7]

  • Surgical resection of bronchiectatic lung or lobectomy: indicated in pulmonary hemorrhage, inviable bronchus, and poor control of symptoms despite optimal medical therapy in unilateral bronchiectasis with well-localized disease
  • Pulmonary artery embolization: indicated in pulmonary hemorrhage
  • Lung transplantation: Consider for severe disease or rapid disease progression.

References: [6]

We list the most important complications. The selection is not exhaustive.

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  3. Chang AB, Bush A, Grimwood K. Bronchiectasis in children: diagnosis and treatment. The Lancet. 2018; 392 (10150): p.866-879. doi: 10.1016/s0140-6736(18)31554-x . | Open in Read by QxMD
  4. Gao Y, Guan W, Zhu Y, Chen R, Zhang G. Antibiotic-resistant Pseudomonas aeruginosa infection in patients with bronchiectasis: prevalence, risk factors and prognostic implications. International Journal of Chronic Obstructive Pulmonary Disease. 2018; Volume 13 : p.237-246. doi: 10.2147/copd.s150250 . | Open in Read by QxMD
  5. Kasper DL, Fauci AS, Hauser S, Longo D, Jameson LJ, Loscalzo J . Harrisons Principles of Internal Medicine . McGraw-Hill Medical Publishing Division ; 2016
  6. T Hill A, L Sullivan A, D Chalmers J, et al. British Thoracic Society Guideline for bronchiectasis in adults. Thorax. 2018; 74 (Suppl 1): p.1-69. doi: 10.1136/thoraxjnl-2018-212463 . | Open in Read by QxMD
  7. Neves PC, Guerra M, Ponce P, Miranda J, Vouga L. Non-cystic fibrosis bronchiectasis. Interact Cardiovasc Thorac Surg. 2011; 13 (6): p.619-625. doi: 10.1510/icvts.2011.284208 . | Open in Read by QxMD
  8. Milliron B, Henry TS, Veeraraghavan S, Little BP. Bronchiectasis: Mechanisms and Imaging Clues of Associated Common and Uncommon Diseases. Radiographics. 2015; 35 (4): p.1011-1030. doi: 10.1148/rg.2015140214 . | Open in Read by QxMD
  9. McShane PJ, Naureckas ET, Tino G, Strek ME. Non–Cystic Fibrosis Bronchiectasis. Am J Respir Crit Care Med. 2013; 188 (6): p.647-656. doi: 10.1164/rccm.201303-0411ci . | Open in Read by QxMD
  10. Polverino E, Goeminne PC, McDonnell MJ, et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J. 2017; 50 (3): p.1700629. doi: 10.1183/13993003.00629-2017 . | Open in Read by QxMD
  11. Máiz L, Nieto R, Cantón R, Gómez G. de la Pedrosa E, Martinez-García M. Fungi in Bronchiectasis: A Concise Review. International Journal of Molecular Sciences. 2018; 19 (1): p.142. doi: 10.3390/ijms19010142 . | Open in Read by QxMD
  12. Maguire G. Bronchiectasis - a guide for primary care. Aust Fam Physician. 2012; 41 (11): p.842-50.
  13. Pasteur MC, Bilton D, Hill AT. British Thoracic Society guideline for non-CFbronchiectasis. Thorax. 2010; 65 (Suppl 1): p.i1-i58. doi: 10.1136/thx.2010.136119 . | Open in Read by QxMD
  14. Hill AT, Barker AF, Bolser DC, et al. Treating Cough Due to Non-CF and CF Bronchiectasis With Nonpharmacological Airway Clearance. Chest. 2018; 153 (4): p.986-993. doi: 10.1016/j.chest.2018.01.014 . | Open in Read by QxMD
  15. 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 (6): p.1084-1092. doi: 10.1111/resp.13047 . | Open in Read by QxMD
  16. Kellett F, Robert NM. Nebulised 7% hypertonic saline improves lung function and quality of life in bronchiectasis. Respir Med. 2011; 105 (12): p.1831-1835. doi: 10.1016/j.rmed.2011.07.019 . | Open in Read by QxMD
  17. Nicolson CHH, Stirling RG, Borg BM, Button BM, Wilson JW, Holland AE. The long term effect of inhaled hypertonic saline 6% in non-cystic fibrosis bronchiectasis. Respir Med. 2012; 106 (5): p.661-667. doi: 10.1016/j.rmed.2011.12.021 . | Open in Read by QxMD
  18. Bilton D, Tino G, Barker AF, et al. Inhaled mannitol for non-cystic fibrosis bronchiectasis: a randomised, controlled trial. Thorax. 2014; 69 (12): p.1073-1079. doi: 10.1136/thoraxjnl-2014-205587 . | Open in Read by QxMD
  19. Chalmers JD, Restrepo MI. Bronchiectasis Management. Chest. 2017; 152 (6): p.1097-1099. doi: 10.1016/j.chest.2017.07.037 . | Open in Read by QxMD
  20. O’Donnell AE, Barker AF, Ilowite JS, Fick RB. Treatment of Idiopathic Bronchiectasis With Aerosolized Recombinant Human DNase I. Chest. 1998; 113 (5): p.1329-1334. doi: 10.1378/chest.113.5.1329 . | Open in Read by QxMD
  21. 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. The Lancet. 2012; 380 (9842): p.660-667. doi: 10.1016/s0140-6736(12)60953-2 . | Open in Read by QxMD
  22. Altenburg J, de Graaff CS, Stienstra Y, et al. Effect of Azithromycin Maintenance Treatment on Infectious Exacerbations Among Patients With Non–Cystic Fibrosis Bronchiectasis. JAMA. 2013; 309 (12): p.1251. doi: 10.1001/jama.2013.1937 . | Open in Read by QxMD
  23. 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 (4): p.e1-e36. doi: 10.1093/cid/ciaa241 . | Open in Read by QxMD
  24. Hill AT, Haworth CS, Aliberti S, et al. Pulmonary exacerbation in adults with bronchiectasis: a consensus definition for clinical research. European Respiratory Journal. 2017; 49 (6): p.1700051. doi: 10.1183/13993003.00051-2017 . | Open in Read by QxMD