Bronchiectasis: Everything you need to know!
What is bronchiectasis?
Bronchiectasis is abnormal, permanent dilatation of bronchi and bronchioles. It can result from either congenital or acquired causes.
What is the mechanism that leads to bronchiectasis?
Specific causes, congenital or acquired, lead to chronic inflammation of the bronchi and bronchioles. This leads to dilatation and thinning of these airways.
What problem is associated with bronchiectasis?
Once there is bronchiectasis, patients have chronic suppurative airway infection with copious sputum production. There is progressive scarring of the affected areas and lung damage occurs.
What are the congenital causes that result in the development of bronchiectasis?
Congenital causes include:
– Cystic fibrosis (commonest)
– Young’s syndrome
– Primary ciliary dyskinesia (Immotile cilia syndrome)
– Kartagener syndrome
What are the acquired causes of bronchiectasis?
Acquired causes include:
– Certain infections during childhood (especially whooping cough & measles)
– Pulmonary tuberculosis
– Inhaled foreign body
– Suppurative pneumonia
– Allergic bronchopulmonary aspergillosis (ABPA)
– Bronchial tumors
– Hypogammaglobinemia
What is the most common cause of bronchiectasis globally?
Pulmonary tuberculosis is the most common cause of bronchiectasis worldwide.
What are the clinical features of bronchiectasis?
The commonest symptom of bronchiectasis is a chronic cough with copious purulent sputum.
A patient may also have hemoptysis, which is usually intermittent streaks of blood but can be larger in amount during infective exacerbations. Massive hemoptysis sometimes occurs requiring bronchial artery embolization.
Other symptoms may include exertional dyspnea & halitosis.
Signs of bronchiectasis include fingernail clubbing. Chest auscultation may reveal coarse mid-inspiratory moist crackles over the affected area, that vanish or change character after cough.
What are the complications that can occur in bronchiectasis?
Complications of bronchiectasis include:
– Infective exacerbations
– Pneumonia
– Massive hemoptysis
– Lobar/lung collapse
– Amyloidosis
– Lung abscess/empyema
How do you diagnose bronchiectasis in a suspected case?
High-resolution CT (HRCT) scan chest is the diagnostic investigation of choice.
Tram-track appearance and signet ring shadows, representing dilated bronchi, longitudinally and in cross-section respectively, are characteristic.
What do you know about different types of bronchiectasis?
There are three types, depending upon the shape of dilated bronchi.
1. Cylindrical or tubular,
2. Varicose, and
3. Cystic.
Tubular bronchiectasis is the most common one.
Bronchiectasis can also be categorized based on the extent, into focal and diffuse.
Focal bronchiectasis indicates local pathology, like bronchiectasis due to a foreign body, due to intrabronchial tumor, or compressing from outside.
Diffuse bronchiectasis indicates systemic disease.
What is traction bronchiectasis?
In certain fibrotic lung diseases or post-TB lung fibrosis, there is a pull or traction on the bronchi arising from parenchymal distortion. This leads to the dilatation of these airways. That’s why the name is given.
Does the location of bronchiectasis have any clinical significance?
Yes, the location of bronchiectasis can give clues regarding the cause.
– Central bronchiectasis – raises the possibility of ABPA.
– Upper lung fields – most commonly involved in cystic fibrosis and post-radiation lung fibrosis.
– Middle lung fields – most likely cause is infection by nontuberculous mycobacteria.
– Lower zones – involved in recurrent aspiration pneumonia (e.g., in scleroderma-associated esophageal dysmotility), traction bronchiectasis secondary to interstitial lung diseases, or recurrent immunodeficiency related lung infections, such as in a patient with hypogammaglobulinemia, is the culprit pathologies.
How will you investigate a patient with bronchiectasis, and what will these investigations reveal?
Chest X-ray – During initial disease, chest x-ray may be normal. In advanced disease, cystic bronchiectasis spaces and thickened airway walls may be visible.
HRCT scan chest – Ct scan is a sensitive modality and diagnostic investigation of choice. It shows tram track and ring shadows signifying dilated and thickened airways.
Sputum culture shall be done during disease exacerbations, for definitive antibiotic treatment.
Spirometry – may reveal an obstructive pattern. If an obstructive pattern is present, perform a reversibility test.
Bronchoscopy – Bronchoscopy can be done for diagnostic as well as therapeutic purposes in patients with hemoptysis.
Other tests: are according to the history, to look for a disease that led to the development of bronchiectasis. The test may include:
– Serum immunoglobulin levels for hypogammaglobinemia.
– Tests for ABPA such as serum precipitins, serum IgE levels, and RAST.
– Tests for cystic fibrosis such as sweat sodium.
What are the main management principles to treat a case of bronchiectasis?
Sputum expectoration
– Efficient airway clearance helps a lot in improving quality of life, and also prevents infective exacerbations and other complications.
– Chest physiotherapy, postural drainage techniques, the use of flutter valve devices, and the use of mucolytic agents all help in sputum expectoration and clearance of the airways.
Antibiotic therapy
– Antibiotics are required for infective exacerbations. These shall be prescribed/adjusted according to the culture sensitivity results.
– Patients known to have Pseudomonas before shall be started empirically on a suitable anti-pseudomonal agent.
– If there are 3 or more infective exacerbations per year, consider long-term antibiotic prophylaxis.
Bronchodilators
Bronchodilators may be required in patients with asthma, COPD, cystic fibrosis, or ABPA.
Treatment of hemoptysis
– Hemoptysis in bronchiectasis often responds to treatment of the underlying infection.
– Percutaneous embolization of bronchial circulation may be needed in the event of massive or repeated hemoptysis.
Surgical treatment
– Excision of bronchiectatic areas is indicated in a minority of cases, in whom bronchiectasis is confined to a single lobe/segment on CT.
– In progressive forms of bronchiectasis, destroyed lung areas that are acting as reservoirs of infection can be resected.
Treatment of the underlying cause
If there is an underlying treatable cause, it must be treated, such as corticosteroids with or without Itraconazole in ABPA.
