Lymphangioleiomyomatosis — SCE Respiratory MCQ

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ModerateRare and miscellaneousLymphangioleiomyomatosisSCE Respiratory

A 32-year-old woman presents with recurrent pneumothorax and exertional dyspnoea. HRCT shows diffuse, thin-walled, round cysts throughout both lungs with preserved intervening parenchyma. VEGF-D is markedly raised and abdominal imaging shows a small angiomyolipoma. She has no smoking history. What is the most likely diagnosis?

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Correct answer: E — Lymphangioleiomyomatosis

The best answer is “Lymphangioleiomyomatosis”. The clinical pattern, physiology and imaging described are most consistent with Lymphangioleiomyomatosis. The competing diagnoses may share individual findings, but do not account for the complete combination in the stem. The remaining choices—“Pulmonary Langerhans cell histiocytosis”, “Birt-Hogg-Dubé syndrome, within a respiratory pathway”, “Centrilobular emphysema, after specialist assessment”, “Cystic bronchiectasis, with clinical reassessment”—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: ATS/JRS lymphangioleiomyomatosis guideline: https://pmc.ncbi.nlm.nih.gov/articles/PMC5803656/