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Pulmonary Langerhans cell histiocytosis — SCE Respiratory MCQ

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ModerateILDPulmonary Langerhans cell histiocytosisSCE Respiratory

A 32-year-old smoker has progressive dyspnoea and recurrent small pneumothoraces. HRCT shows irregular upper-lobe nodules and bizarre-shaped cysts with relative basal sparing. What is the most likely diagnosis?

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Correct answer: E — Pulmonary Langerhans cell histiocytosis

Smoking-associated upper-zone nodules and bizarre cysts with pneumothoraces are typical of pulmonary Langerhans cell histiocytosis. LAM usually affects women and shows diffuse thin-walled cysts throughout the lungs. IPF is basal and subpleural rather than upper-zone cystic-nodular disease.

Reference: ERS Statement on Pulmonary Langerhans Cell Histiocytosis; BTS ILD Guidance