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CF Bronchiectasis — RACP Adult Medicine MCQ

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EasyRespiratoryCF BronchiectasisRACP Adult Medicine

A 30-year-old man with cystic fibrosis presents with recurrent productive cough and HRCT showing bilateral upper-lobe predominant bronchiectasis with mucus plugging. Sputum grows Pseudomonas aeruginosa. Sweat chloride is 85 mmol/L. What is the most likely aetiology of bronchiectasis?

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Correct answer: A — Cystic fibrosis

Bilateral upper-lobe predominant bronchiectasis with chronic Pseudomonas colonisation and elevated sweat chloride (>60 mmol/L diagnostic) confirms CF-related bronchiectasis. CFTR modulator therapy (elexacaftor/tezacaftor/ivacaftor for patients with at least one F508del allele) has transformed CF management, significantly improving FEV₁ and reducing exacerbation rates.

Reference: CF Australia – 2024 – CF Guidelines; eTG Respiratory 2024