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General Medicine · Respiratory medicine: pulmonary alveolar proteinosis
Sample itemA 38-year-old man has had progressive exertional breathlessness and a dry cough for 8 months, with mild hypoxaemia. HRCT of the chest shows ground-glass opacities with smooth interlobular septal thickening in a polygonal pattern (crazy paving). Bronchoalveolar lavage returns milky fluid containing PAS-positive lipoproteinaceous material. What is the most appropriate treatment?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
B. Whole-lung lavage
Pulmonary alveolar proteinosis is usually autoimmune, caused by antibodies to GM-CSF that impair alveolar macrophage clearance of surfactant. The treatment for symptomatic or hypoxaemic disease is therapeutic whole-lung lavage under general anaesthesia, with inhaled GM-CSF as an emerging option. Corticosteroids are ineffective and raise the risk of opportunistic infections such as Nocardia.
Option by option
A. Empirical antituberculosis therapy
Antituberculosis therapy treats tuberculosis; the milky PAS-positive lavage points to proteinosis, not infection.
B. Whole-lung lavage (correct)
Whole-lung lavage physically removes the accumulated surfactant-rich material and improves oxygenation.
C. Prolonged high-dose prednisolone
Corticosteroids do not help alveolar proteinosis and increase infection risk; they suit organising pneumonia or sarcoidosis.
D. Pirfenidone
Pirfenidone is an antifibrotic for idiopathic pulmonary fibrosis, which does not give milky lavage fluid.
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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