Bronchiectasis and CF

Question 1

What is bronchiectasis?

Answer 1

• Permanent dilatation of the airways
• Secondary to chronic infection or inflammation
• Wide variety of causes

Question 2

What are congenital causes of bronchiectasis?

Answer 2

• Cystic fibrosis → most common
• Kartagener’s syndrome
• Young’s syndrome

Question 3

What are post-infective causes of bronchiectasis?

Answer 3

• Measles
• Pertussis
• TB
• Pneumonia
• Bronchiolitis

Other causes → UC / RA / bronchial obstruction

Question 4

What are the clinical features of bronchiectasis?

Answer 4

• Persistent cough
• Copioid purulent sputum
• Intermittent haemoptysis
• Signs → clubbing / coarse insp creps / wheeze

Question 5

What are complications of bronchiectasis?

Answer 5

• Pneumonia
• Pleural effusion
• Pneumothorax
• Amyloidosis

Question 6

What may sputum culture show for bronchiectasis?

Answer 6

• Single or multiple pathogens present
• Most common (25%) = gram-negative → pseudomonas aeruginosa

Question 7

What other investigations are done for bronchiectasis?

Answer 7

• CXR → cystic shadows / thickened bronchial walls
• CT → extent + distribution / dilated bronchus wider than accompanying pulm artery + resemble signet ring
• Spirometry → obstructive pattern
• Bronchoscopy → site of haemoptysis / exclude obstruction / obtain culture samples
• Other tests → serum Igs / CF sweat test / Aspergillus preceiptans or skin-prick test RAST + total IgE

Question 8

What is the management of bronchiectasis?

Answer 8

• Airway clearance → chest physio / flutter valve
• ABx → known pseudomonas: oral ciprofloxacin or suitable IV abx
• Bronchodilators → useful in asthma, COPD, CF, ABPA
• Corticosteroids → prednisolone and itraconazole for ABPA
• Surgery → indicated in localised disease or to control severe haemoptysis