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Bronchiectasis

Essentials

  • Bronchiectasis means permanent enlargement of the bronchi and chronic inflammation of the bronchial walls and the structures surrounding them.
  • 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
    • immunodeficiency
    • sequelae of infectious diseases: bacterial infections, mycobacterial infections (tbc, environmental mycobacteria), whooping cough, viral diseases (measles, adenovirus, influenza)
    • mechanical obstruction (foreign body, stenosis, tumour, lymph node)
    • toxic damage (gastro-oesophageal reflux, aspiration, toxic inhalation)
    • obstructive pulmonary diseases (asthma, COPD) and alpha1-antitrypsin deficiency
    • systemic diseases (rheumatoid arthritis, connective tissue disorders, inflammatory bowel diseases).
  • 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.

Examinations in primary health care

  • ESR, CRP, complete blood count
  • Imaging of the maxillary sinuses
  • Spirometry (mild irreversible constriction common)

Aetiological examinations in specialized care

  • High-resolution computed tomography
  • Bronchoscopy, as necessary
  • Sweat test and CFTR gene test at least in patients below the age of 40 years
  • Immunoglobulins
  • Complete blood count and, at least in lymphopenic patients, B-cell immunophenotyping
  • Vaccination response
  • Rheumatoid factor, antinuclear antibody and ENA antibody levels
  • Alpha1-antitrypsin if chest x-ray suggests emphysema
  • Aspergillus IgG and IgE
  • Examination of ciliary function

Treatment Inhaled Hyperosmolar Agents for Bronchiectasis, Pneumococcal Vaccines for Bronchiectasis, Influenza Vaccine for Children and Adults with Bronchiectasis, Inspiratory Muscle Training for Bronchiectasis, Antibiotics for Purulent Bronchiectasis, Mucolytics for Non-Cystic Fibrosis Bronchiectasis, Inhaled Steroids for Bronchiectasis, Nurse Specialist Care for Bronchiectasis, Surgery for Bronchiectasias

Conservative

  • Physical therapy
    • 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.
  • Influenza and pneumococcal vaccination

Surgical treatment

  • Indications
    • Persistent symptoms despite conservative treatment
    • Lesions in a circumscribed area
  • Surgical treatment is seldom used these days.

Treatment chain

  • Diagnosis in specialized care
  • 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, et al. 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]