Case 7.4 – Bronchiectasis [SDL]

History:

The history must include an assessment of the duration of symptoms, including the appearance of and volume of sputum, and associated respiratory symptoms of chest pain and shortness of breath.

Systemic symptoms such as fever, malaise, nausea, vomiting, weight loss and anorexia should be sought.

Check past medical history including intercurrent illness, past history of smoking, past medical illnesses, travel and occupational history.

Physical Examination:

Physical examination includes vital signs and pulse oximetry. It needs to include an assessment of nutritional status. Examine for fever, cachexia, and peripheral cyanosis.

Clubbing may be present if chronic. Ask to view a sputum sample.

Examination of the chest should reveal coarse crackles all over affected lobe.

Sputum Examination (microscopy, examination, sensitivity):

In addition to common respiratory pathogens, sputum culture may reveal Pseudomonas aeruginosa, fungi such as Aspergillus and various Mycobacteria.

Frequent cultures are necessary to ensure appropriate treatment of resistant organisms.

Radiology:

• Bronchiectasis, unless very gross, is not usually apparent on a chest x-ray
• In advanced disease, thickened airway walls, cystic bronchiectatic spaces, and associated areas of pneumonic consolidation or collapse may be visible
• CT is much more sensitive to show thickened dilated airways

Assessment of Ciliary Function:

A screening test can be performed in patients suspected of having a ciliary dysfunction syndrome by assessing the time taken for a small pellet of saccharin placed in the anterior chamber of the nose to reach the pharynx, when the patient can taste it. This time should not exceed 20 minutes and is greatly prolonged in patients with ciliary dysfunction. Ciliary beat frequency may also be assessed using biopsies taken from the nose. Structural abnormalities of cilia can be detected by electron microscopy.

Symptoms due to accumulation of pus in dilated bronchi:

• Chronic productive cough usually worse in mornings and often brought on by changes of posture
• Sputum often copious and persistently purulent in advanced disease
• Halitosis is a common accompanying feature

Symptoms due to inflammatory changes in lung and pleura surrounding dilated bronchi:

• Fever
• Malaise
• Increased cough and sputum volume when spread of infection causes pneumonia, which may be associated with pleurisy
• Recurrent pleurisy in the same site often occurs in bronchiectasis

Haemoptysis:

• Can be slight or massive and is often recurrent
• Usually associated with purulent sputum or an increase in sputum purulence

Systemic symptoms:

When disease is extensive and sputum persistently purulent a decline in general health occurs with weight loss, anorexia, lassitude, low-grade fever, and failure to thrive in children. In these patients digital clubbing is common.

Congenital:

• Cystic fibrosis
• Ciliary dysfunction syndromes
• Primary ciliary dyskinesia (immotile cilia syndrome)
• Kartagener's syndrome (sinusitis and transposition of the viscera)
• Primary hypogammaglobulinaemia

Acquired (children):

• Pneumonia (complicating whooping cough or measles)
• Primary TB
• Inhaled foreign body

Acquired (adults):

• Suppurative pneumonia
• Pulmonary TB
• Allergic bronchopulmonary aspergillosis complicating asthma
• Bronchial tumours

Physiotherapy:

Patients should be instructed how to perform regular daily physiotherapy to keep the dilated bronchi empty of secretions. When this is performed correctly, it is highly effective to reduce the cough and amount of sputum produced. This may help to prevent recurrent episodes of bronchopulmonary infection.

• Patients should adopt a position in which the lobe to be drained is uppermost
• Deep breathing followed by forced expiratory manoeuvres is of help in allowing secretions in the dilated bronchi to gravitate towards the trachea, from which they can be cleared by vigorous coughing
• 'Percussion' of the chest wall with cupped hands may help to dislodge sputum, and a number of mechanical devices are available which cause the chest wall to oscillate, thus achieving the same effect
• This all needs to be undertaken for 5-10 minutes once or twice daily at a minimum for most patients

Antibiotics:

This may be difficult due to organisms such as staphylococci and Gram-negative bacilli, in particular Pseudomonas species. In these circumstances antibiotic therapy should be guided by the microbiological results but frequently requires the use of oral ciprofloxacin (250-750 mg 12-hourly) or ceftazidime by intravenous injection or infusion (1-2 g 8-hourly).

Surgery:

Very few people are suitable for surgery. These are most often young people in whom the bronchiectasis is unilateral and confined to a single lobe or segment as demonstrated by CT. There are high rates of problems as there is often co-incidental areas of damaged lungs acting as reservoirs of infection.