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General Medicine · Respiratory medicine: pulmonary Langerhans cell histiocytosis
Sample itemA 28-year-old heavy smoker has a dry cough, dyspnoea and a spontaneous pneumothorax. HRCT shows bizarre-shaped cysts and nodules in the upper and mid zones with sparing of the costophrenic angles. Biopsy shows cells with Birbeck granules. What is the most important first step in management?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Smoking cessation
Pulmonary Langerhans cell histiocytosis is strongly linked to cigarette smoking, and stopping smoking can lead to stabilisation or regression of disease. Immunosuppression is reserved for refractory or progressive disease and transplantation for end-stage disease.
Option by option
A. Pulsed cyclophosphamide
Cytotoxic therapy is for refractory disease, not an initial step.
B. A prolonged course of oral prednisolone
Steroids have no proven benefit as first-line therapy.
C. Smoking cessation (correct)
Correct. Smoking cessation is the cornerstone and may lead to regression.
D. Referral for lung transplantation
Transplantation is for advanced respiratory failure.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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