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Pulmonary Langerhans cell histiocytosis — SCE Respiratory MCQ

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ModerateInterstitial Lung DiseasePulmonary Langerhans cell histiocytosisSCE Respiratory

A 41-year-old smoker has progressive dyspnoea and cough. HRCT shows multiple irregular upper-zone cysts and small nodules with sparing of the costophrenic angles. Lung biopsy shows CD1a-positive Langerhans cells. What is the most appropriate management?

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Correct answer: CSmoking cessation with specialist ILD follow-up

Pulmonary Langerhans cell histiocytosis is strongly smoking-associated, and smoking cessation is the key initial intervention with ILD follow-up. Inhaled corticosteroids do not alter disease biology. TB or fungal therapy would not treat biopsy-proven PLCH, and transplantation is reserved for advanced progressive disease.

Reference: ERS/BTS ILD resources; BTS Smoking Cessation resources