Lymphangioleiomyomatosis — SCE Respiratory MCQ

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HardRare/MiscellaneousLymphangioleiomyomatosisSCE Respiratory

A 36-year-old woman of childbearing age has recurrent pneumothoraces and progressive dyspnoea. HRCT shows diffuse, round, thin-walled lung cysts with normal intervening lung. Serum VEGF-D is raised. What is the most likely diagnosis?

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Correct answer: D — 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”, “Idiopathic pulmonary fibrosis”, “Chronic hypersensitivity pneumonitis”, “COPD, after safety review”—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/