Bronchiectasis should be kept in mind as a possible cause of prolonged or recurrent respiratory infections, particularly in adults with prolonged cough, recurring paranasal sinusitis or otitis media.
In patients with bronchiectasis, always examine whether there is a history of severe respiratory tract infections.
Aetiology
Conditions associated with bronchiectasis include:
ciliary dysfunction, such as cystic fibrosis Cystic Fibrosis (CF), primary ciliary dyskinesia
In 25 to 50% of patients, no cause is found for the disease even after extensive investigation.
Symptoms and findings
Symptoms
Copious sputum production (particularly in the absence of a history of smoking), cough, dyspnoea, haemoptysis
Recurrent bronchitis and pneumonia
Recurrent fever associated with respiratory tract symptoms
Signs
Coarse crackles (audio sample Bronchiectasia), or normal auscultation findings
Sometimes expiratory wheezing
Diagnosis
Chest x-rays are often normal. Increased peribronchial markings and a honeycomb pattern may be seen.
High-resolution computed tomography (HRCT)
Will confirm the diagnosis.
The bronchial diameter is larger than the diameter of the adjacent artery, the bronchial walls are thickened, and the normal tapering of the bronchi towards the periphery of the lungs is absent.
Sputum samples should be examined at follow-up visits and in connection with any exacerbation to find out if there is microbial colonization (bacteria, fungi, tuberculosis).
If a person less than 30-40 years of age contracts environmental mycobacteriosis or if several of the patient's relatives have had such a disease, the patient should be referred for assessment to a physician with special expertise in immunodeficiencies. See Pulmonary Diseases Caused by Environmental Mycobacteria.
All patients with bronchiectasis should receive instruction from a physiotherapist on how to use postural drainage aids and on breathing techniques. Postural drainage at home, blowing into a PEP bottle Chronic Obstructive Pulmonary Disease (COPD) or other draining device based on counterpressure, strengthening of inspiratory muscles with the help of a dedicated device and exercising until breathless may be useful.
Active fitness training
Antimicrobial treatment in association with any exacerbations and if the patient has copious purulent sputum
The most significant infective bacteria are Streptococcus pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, Pseudomonas aeruginosa and Staphylococcus aureus.
If no bacterial sample is available, the first choice antimicrobial drugs are amoxicillin or a combination of amoxicillin and clavulanic acid, for patients with penicillin allergy doxycycline. 14-day courses are recommended.
Quinolones should be reserved for the treatment of Pseudomonas infections.
Sputum colonization samples should be examined at follow-up visits and, as far as possible, also in association with any exacerbation before starting antimicrobial treatment.
Long-term antimicrobial treatment can be considered if there are at least three exacerbations per year. The drug should be chosen based on antimicrobial susceptibility but ciprofloxacin should not be used.
Azithromycin has been shown to be useful but its efficacy is primarily based on its anti-inflammatory effect. Azithromycin is suitable for the prophylaxis of recurrent exacerbations even when a specific bacterial colonization cannot be identified. If exacerbations continue to recur (3 or more per year), azithromycin can be combined with an inhaled antimicrobial drug.
Inhaled antimicrobial drugs (tobramycin, colistin) can be used in chronic Pseudomonas colonization, in particular, even though there is no actual research-based evidence on their usefulness other than in patients with cystic fibrosis.
Inhaled hypertonic saline may be useful. It reduces the viscosity of the mucus, facilitates coughing it up and improves lung function and quality of life.
There is not enough scientific evidence for routine use of mucolytics in bronchiectasis.
Beta2-sympathomimetics and tiotropium are used to treat airway obstruction in patients with bronchiectasis. However, there is no research-based evidence to support this therapeutic practice.
After diagnostic examinations, patients can be monitored in primary health care if their disease is stable and there are no recurrent exacerbations.
Clinical follow-up and blood tests to detect inflammation, as considered individually necessary; sputum colonization sample, if possible, spirometry and chest x-ray
After diagnosis, patients with any of the following should be treated in specialized care:
recurrent growth of Pseudomonas, methicillin-resistant Staphylococcus aureus or environmental mycobacteria in sputum
recurrent exacerbations (more than 3/year)
progressive disease and deteriorating lung function
bronchiectasis associated with any of the following: allergic bronchopulmonary aspergillosis, aspergilloma, rheumatoid arthritis, immunodeficiency, inflammatory bowel disease or ciliary dysfunction.
Prognosis
In countries with appropriate health services, the disease hardly affects the life expectancy of the patients.
References
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):660-7. [PubMed]
Hill AT, Sullivan AL, Chalmers JD ym. British Thoracic Society Guideline for bronchiectasis in adults. Thorax 2019;74(Suppl 1):1-69. [PubMed]
Polverino E, Goeminne PC, McDonnell MJ et al. European Respiratory Society guidelines for the management of adult bronchiectasis. Eur Respir J 2017;50(3):. [PubMed]