Table of Contents
What is Bronchiectasis?
Bronchiectasis is abnormal and permanent dilatation of the bronchi. The trachea divides into two to give rise to bronchi. These bronchi become increased in sized (dilated) in Bronchiectasis. When the bronchi become dilated (widened), they cause chronic and recurrent infections of the airways and secretions pool within it.
Bronchiectasis gross pathology is the dilatation of the bronchi. This occurs through a vicious cycle of transmural infection and inflammation with release of mediators of inflammation such as elastase, interleukin 8, tumor necrosis factor alpha (TNF-alpha) and prostanoids.
The release of these mediators of inflammation causes more and more damage of the bronchi leading to increasing dilation.
Bronchiectasis Causes (Etiology)
- Bacterial infections such as pseudomonas infections and haemophilus infection
- Mycobacteria tuberculosis infection
- Aspergillus species infection
- Viral infections such as adenovirus, measles, influenza virus and HIV
- Primary ciliary dyskinesia
- Cystic fibrosis
- Pulmonary sequestration
- Marfans syndrome
- Cartilage (Williams-Cambell syndrome)
- Tracheobronchomegaly (Mounier-Kulin syndrome)
- Immunodeficiency causes of Bronchiectasis such as hypogammaglobinemia,
- Post transplantation
- Toxic inhalation from chlorine
- Toxicity due to overdose of drugs such as heroin
- Foreign bodies in the airways
- Rheumatoid arthritis
- Systemic lupus erythematosus (SLE)
- Sjogrens syndrome
- Relapsing polychondritis
- Inflammatory bowel disease
- Yellow nail syndrome
- Recurrent cough with purulent sputum
- Hemoptysis (coughing out of blood) this occurs in about 50 to 70% of patients
- It may show no symptoms (asymptomatic)
- If it is dry Bronchiectasis occurring in the upper lobe of the lungs, there may be non-productive cough
- Wheezing may occur especially when there is widespread Bronchiectasis. Wheezing may occur also when there is accompanying Chronic Obstructive Pulmonary Disease (COPD)
- When there is exacerbations, the amount of sputum increases, becomes more purulent and most times bloody. There may be fever.
- Crackles in the lungs
- Rhonchi and wheeze may be heard in the lungs
- Digital finger clubbing may occur
- Chronic hypoxemia may cause right heart failure with its symptoms and signs
Numbers 1 to 4 are the post-infectious causes of Bronchiectasis (that is, Bronchiectasis usually occurs after the infections).
Number 6 to 10 are the congenital causes of Bronchiectasis that are due to alpha 1 antitrypsin deficiency.
Bronchiectasis X Ray Features
- Chest X ray findings may be normal at one extreme. In the secular type of Bronchiectasis, air fluid levels may be seen
- Dilated airways with thickened bronchiolar walls may be seen
Bronchiectasis Diagnosis and Laboratory Investigations
- CT scan has now replaced bronchography for visualization of the airway and CT scan is now the investigation of choice in the diagnosis of Bronchiectasis.
- Sputum microscopy, culture and sensitivity
- Sweat chloride level may be increased in cystic fibrosis
- Structural and functional assessment of nasal and bronchial cilia or sperm in primary ciliary dyskinesia
- There may be decreased level of immunoglobulins which may be the cause of the recurrent infection
- Lung function tests may show obstructive patterns in diffuse Bronchiectasis or in association with COPD
Bronchiectasis Treatment Guidelines
- The aim of treatment in Bronchiectasis is: to eliminate the underlying cause of the Bronchiectasis
- Improvement in clearance of the tracheobronchial tree
- Control of infections
- Reversal of airway obstruction
Bronchiectasis Treatment Guidelines for specific causes
- Gammaglobulin for hypogammaglobulinemia
- Use of anti-tuberculous drugs if tuberculosis is the cause
- Use of glucocorticoids in allergic bronchopulmonary aspergillosis (ABPA)
- Use of aerosol of recombinant DNAse helps in cystic fibrosis
- Use of appropriate antibiotics from culture results
- Chest physical therapy with vibration, percussion and postural drainage may be helpful
- Bronchodilators are helpful in patients with airway hyperactivity and reversible obstruction
- Surgery may be indicated
Indications for Surgery in Bronchiectasis
- Localized lesion
- Failure of medical therapy
- Massive coughing of blood
- Lung transplant for patents who are disabled despite maximum therapy
- Metastatic cerebral abscess
- Severe, life-threatening hemoptysis can occur, especially in those patients with cystic fibrosis.
- Bronchiectasis caused by congenital causes such as in cystic fibrosis or Marfans syndrome or any other congenital cause generally has a bad prognosis compared with those of acquired type.
- Smoking when you have bronchiectasis has a bad prognosis
- Frequent exacerbations have bad prognosis
- Extensive involvement of both bronchi
Bronchiectasis Life Expectancy
Many of those having Bronchiectasis have a normal life expectancy except in severe cases of Bronchiectasis having bad prognosis.