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Pulmonary alveolar proteinosis — SCE Respiratory MCQ

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HardILDPulmonary alveolar proteinosisSCE Respiratory

A 44-year-old man has progressive breathlessness and a crazy-paving pattern on HRCT. Bronchoalveolar lavage returns opaque milky fluid that is PAS positive; GM-CSF autoantibody is positive. What is the most appropriate management?

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Correct answer: B — Whole-lung lavage in a specialist centre

Autoimmune pulmonary alveolar proteinosis with significant symptoms is treated with whole-lung lavage in an experienced centre. Antibiotics or TB treatment do not address surfactant accumulation. Pleurodesis is unrelated unless recurrent pleural air or fluid is present.

Reference: ERS Guidelines on Pulmonary Alveolar Proteinosis; BTS ILD Guidance