Bronchiectasis

Question 1

define bronchiectasis

Answer 1

Lung airway disease characterized by chronic bronchial dilation, impaired mucuociliary clearance and frequent bacterial infections.

Question 2

aeitiology of bronchiectasis

Answer 2

severe inflammation of bronchi and bronchioles = fibrosis and dilation and thinning

followed by pooling of mucus, predisposing to further cycles of infection, damage and fibrosis to bronchial walls

causes:

• idiopathic 50%
• post-infectious - After severe pneumonia, whooping cough, tuberculosis, measles, pertussis, bronchiolitis, HIV. - H. influenzae; Strep. pneumoniae; Staph. aureus; Pseudomonas aeruginosa.
• Host defence defects: e.g. Kartagener’s syndrome, cystic fibrosis, immunoglobulin deficiency, yellow-nail syndrome, Young’s syndrome, primary ciliary dyskinesia
• obstruction of bronchi - foreign body, enlarged lymph nodes, tumour
• Congenital/Hereditary: Cystic fibrosis, Ciliary dyskinesia, Marfan’s syndrome, alpha1-antitrypsin deficiency
• inhalation/aspiration
• allergic bronchopulmonary aspergillosis
• pul fibrosis
• gastric reflux disease
• inflamm disorders - rheumatoid arthritis, UC
• immunodeficiency states - AIDS usually as a result of recurrent infection
• hypogammaglobulinaemia
• sarcoidosis
• bronchiolitis obliterans

Question 3

epidemiology of bronchiectasis

Answer 3

Most often arises initially in childhood, incidence has reduced with use of antibiotics, approximately 1 in 1000 per year.

Question 4

presenting symptoms of bronchiectisis

Answer 4

cough with purulent sputum or haemoptysis

SOB

chest pain

malaise

fever

weight loss

symptoms usually begin after acute resp distress

Question 5

signs of bronchiectasis

Answer 5

finger clubbing

coarse inspiratory crepitations (usually at bases), shift with coughing

wheeze - asthma, COPD, ABPA

Question 6

investigations for bronchiectasis

Answer 6

sputum

• culture and sensitivity
• common organisms: Pseudomonas aeruginosa, Haemophilus influenzae, Staphylococcus aureus, Streptococcus pneumoniae, Klebsiella, Moraxella catarrhalis, Mycobacteria.

CXR

• dilated bronchi (thickened bronchial walls) seen as parallel lines from hilum to diaphragm (tramline shadows)
• fibrosis
• atelectasis
• pneumonic consolidations
• may be normal
• cystic shadows

high resolution CT chest

• dilated bronchi with thick walls
• best diagnostic method

bronchoscopy

• locate site of haemoptysis
• exclude obstruction
• obtain samples for culture

bronchography (rarely used)

• determine extent of disease before surgery
• radio-opaque contrast injected through the cricoid ligament or via a bronchoscope).

sweat electrolytes

serum Ig - 10% adults have some immune deficiency

sinus XR - 30% have concomitant rhinosinusitis

mucociliary clearance study

Aspergillus precipitins or skin-prick test RAST and total IgE.

spirometry

• obstructive pattern
• reversibility should be assessed

Question 7

CXR changes with bronchiectasis

Answer 7

thickened bronchial walls

ring shadows - thickened airways seen end on

volume loss due to mucus plugging

air fluid levels may be visible in dilated bronchi

Question 8

bronchiectasis management

Answer 8

acute exacerbations

• 2 IV AB or oral ciprofloxacin with efficacy for pseudomonas
• prophylactic course of AB (oral or aerosolized) for those with frequent >3/yr exacerbations

inhaled corticosteroids eg fluticasone

• reduce inflammation and volume of sputum
• doesnt affect freq of exacerbations or lung function
• bronchodilators - considered in pts with responsive disease
• for ABPA

bronchodilators

• eg nebulised salbutamol
• useful in pts with asthma, COPD, CF, ABPA

maintain hydration - adequate oral intake

consider flu vaccine

physio

• Cornerstone of management is sputum and mucus clearance techniques (e.g.postural drainage).
• shown how to position themselves so lobe to be drained is uppermost
• for 20mins twice daily
• reduce freq of acute exacerbations and aids recovery

flutter valve may aid sputum expectoration and mucus drainage

mucolytics

bronchial artery embolisation - life threatening haemoptysis due to bronchiectasis

surgical for localised disease or haemoptysis - localized resection, lung or heart–lung transplantation.

Question 9

complications of bronchiectasis

Answer 9

life threatening haemoptysis

persistent infections

pneumonia

pleural effusion

pneumothorax

empyema

respiratory failure

cor pulmonale

multi-organ abscesses (cerebral)

amyloidosis

Question 10

prognosis for pts with bronchiectasis

Answer 10

Most patients continue to have the symptoms after 10 years.