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Lymphangioleiomyomatosis — SCE Respiratory MCQ

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HardRare respiratory diseaseLymphangioleiomyomatosisSCE Respiratory

A 32-year-old non-smoking woman presents with recurrent spontaneous pneumothorax and progressive exertional dyspnoea. HRCT shows diffuse, thin-walled, round cysts distributed throughout otherwise normal lung; renal ultrasound shows a 4 cm angiomyolipoma. What is the most appropriate investigation?

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Correct answer: E — Serum vascular endothelial growth factor-D level

The best answer is “Serum vascular endothelial growth factor-D level”. Serum vascular endothelial growth factor-D level is the investigation that most directly resolves the remaining diagnostic or staging uncertainty at this point in the pathway. The alternatives address different questions, have lower expected yield, or would be premature before this result. The remaining choices—“Bronchial challenge testing, within a respiratory pathway”, “Pleural fluid adenosine deaminase”, “Aspergillus-specific IgE, with clinical reassessment”, “Transbronchial needle aspiration of hilar nodes”—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 pleural disease: spontaneous pneumothorax: https://thorax.bmj.com/content/78/Suppl_3/s1