(Clinical Imaging)

Overview

• Permanent dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall.
• Classfied as either CF bronchiectasis or non-CF bronchiectasis
• Aetiology → recurrent pulmonary infections, cystic fibrosis, tuberculosis, COPD, aspiration, immunodeficiency, chronic inflammatory diseases (RA, CD), primary ciliary dyskinesia, allergic bronchopulmonary aspergillosis, A1AT deficiency
  • Cystic Fibrosis ⇒ autosomal recessive disorder causing increased viscosity of secretions. Due to a defect in the the cystic fibrosis transmembrane conductance regulator gene (CFTR), which codes a chloride channel. May cause recurrent chest infections, failure to thrive (delayed puberty), malabsorption (steatorrhoea), short stature, male infertility. Diagnosed via sweat test (will have abnormally high sweat chloride). Typically screened at birth via heel prick test. Mx involves chest physiotherapy.
  • Primary Ciliary Dyskinesia (Kartagener’s Syndrome) ⇒ bronchiectasis + dextrocardia (or complete sinus inversus = right testicle hanging lower than left) + recurrent sinusitis. Autosomal recessive disorder. Can also cause male infertility.
  • Allergic Bronchopulmonary Aspergillosis ⇒ bronchiectasis + bronchoconstriction (wheeze, cough, dyspnoea - patient may have previous label of asthma). Will also have eosinophilia and raised IgE. Tx with oral prednisolone. Hypersensitivity response to the fungus aspergillus.
• Obstructive disease → mucus plugs form in airways that obstruct airflow

Making Diagnosis

Clinical Features:

• Chronic Cough (months-years) → associated with large (copious) amounts of purulent sputum (green/rusty colour). May be worsened by lying flat or on one side.
• Sputum → may be bloody (haemoptysis) in 50% of patients and is usually mild
• Recurrent chest infections
• Clubbing
• Inspiratory coarse crackles on auscultation
• SOB → especially on exertion
• Fever → acute exacerbation often presents with
• Fatigue, Weight Loss, Wheezing