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Lymphangioleiomyomatosis — SCE Respiratory MCQ

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HardOtherLymphangioleiomyomatosisSCE Respiratory

A 35-year-old woman with known pulmonary lymphangioleiomyomatosis has worsening dyspnoea. HRCT shows diffuse thin-walled cysts and renal imaging shows a 5 cm angiomyolipoma. FEV1 has fallen from 82% to 64% predicted over 18 months and VEGF-D is elevated. What is the most appropriate management?

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Correct answer: D — Commence sirolimus after specialist assessment

Progressive LAM with declining lung function and a significant angiomyolipoma is an indication to consider mTOR inhibition such as sirolimus in a specialist setting. Corticosteroids and inhaled asthma therapy do not modify LAM progression. Pleural surgery is reserved for recurrent pneumothorax management, and pirfenidone is used in fibrotic ILD rather than LAM.

Reference: ERS LAM Guideline; BTS Pleural Disease Guideline 2023