Bronchiectasis

Question 1

Define bronchiectasis

Answer 1

the permanent dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall with frequent bacterial infections

Question 2

What are the causes/risk factors of bronchiectasis?

Answer 2

• Idiopathic (50%)
• Post-infection e.g. pneumonia, whooping cough, TB
• Genetic e.g. Kartagener’s syndrome (ciliary dyskinesia, situs invertus, chronic
sinusitis), cystic fibrosis, alpha-1 antitrypsin deficiency
• Connective tissue disorders e.g. RA
• Inflammatory bowel disease e.g. UC, Crohn’s
• Bronchial obstruction e.g. foreign body, tumour

Question 3

What are the presenting symptoms of bronchiectasis?

Answer 3

• Productive cough
• Purulent sputum
• Haemoptysis
• SOB
• Pleuritic chest pain
• Malaise
• Fever
• Weight loss

Question 4

What are the signs of bronchiectasis?

Answer 4

• Clubbing
• Coarse crepitations – high-pitched inspiratory squeaks and pops
• Wheeze
• Signet ring sign
• Tram tracks

Question 5

What are the investigations for bronchiectasis?

Answer 5

• CXR - characteristic volume loss, hemidiaphragm, tram lines, tubular/ovoid opacisites
• High res CT - thickened, dilated airways with or without air fluid levels; varicose constrictions along airways; cysts and/or tree-in-bud pattern
• FBC - high eosinophil count in bronchopulmonary aspergillosis; neutrophilia suggests superimposed infection or exacerbation
• sputum culture and sensitivity
• serum alpha-1 antitrypsin phenotype and level
• serum immunoglobulin - decreased IgG, IgM, and/or IgA with inappropriate titres to Pneumovax
• sweat chloride test - >60 mmol/L (>60 mEq/L) cystic fibrosis is likely; 40 to 59 mmol/L cystic fibrosis is possible; <39 mmol/L cystic fibrosis is very unlikely
• rheumatoid factor
• aspergillus fumigatus skin prick test
• serum HIV antibody
• nasal nitric oxide - low (<100 parts per billion) NNO level in primary ciliary dyskinesia; high NNO level excludes a diagnosis of PCD
• pulmonary function test - reduced FEV1, elevated RV/TLC

Question 6

What is the management plan for bronchiectasis?

What potential complications can arise?

Answer 6

maintenance therapy and treatment of acute exacerbations, with attention to the specific organism involved

For a select group of patients, surgery may be indicated

Patients with severe disease or a resistant organism (typically Pseudomonas) are likely to require IV antibiotics during acute exacerbations

For severe, progressive disease lung transplantation should be considered

complications:
- massive haemoptysis - variable timeframe and low likelihood
- respiratory failure - variable timeframe and low likelihood
- cor pulmonale - variable timeframe and low likelihood