Primary ciliary dyskinesia — SCE Respiratory MCQ

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HardRare/MiscellaneousPrimary ciliary dyskinesiaSCE Respiratory

A 58-year-old woman has chronic productive cough, sinusitis and infertility. CT shows diffuse bronchiectasis, most marked in the middle and lower lobes. Nasal nitric oxide is very low and ciliary beat studies are abnormal. What is the most likely underlying cause?

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Correct answer: D — Primary ciliary dyskinesia

The best answer is “Primary ciliary dyskinesia”. The clinical pattern, physiology and imaging described are most consistent with Primary ciliary dyskinesia. The competing diagnoses may share individual findings, but do not account for the complete combination in the stem. The remaining choices—“Smoking-related COPD, after specialist assessment”, “Alpha-1 antitrypsin deficiency”, “Allergic bronchopulmonary aspergillosis”, “Idiopathic pulmonary fibrosis”—are credible in related respiratory presentations, but each addresses a different diagnostic, staging or management decision and does not fit the decisive findings and pathway position in this stem.

Reference: British Thoracic Society guideline for bronchiectasis in adults: https://thorax.bmj.com/content/74/Suppl_1/1