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Pulmonary Langerhans cell histiocytosis — SCE Respiratory MCQ

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HardILDPulmonary Langerhans cell histiocytosisSCE Respiratory

A 35-year-old smoker presents with cough and exertional dyspnoea. HRCT shows upper-lobe nodules and bizarre-shaped cysts with sparing of the costophrenic angles. Spirometry is near normal but TLCO is 55% predicted. What is the most appropriate management?

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Correct answer: A — Smoking cessation with respiratory follow-up

The HRCT pattern in a smoker is classic for pulmonary Langerhans cell histiocytosis, and smoking cessation is the key intervention. Steroids are not routine first-line treatment for typical mild disease. TB therapy and endobronchial valves do not address the underlying smoking-related cystic lung disease.

Reference: ERS Rare ILD guidance; BTS ILD guidance