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Pulmonary Alveolar Proteinosis — RACP Paediatrics MCQ

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HardRespiratoryPulmonary Alveolar ProteinosisRACP Paediatrics

A previously well 3-year-old develops progressive tachypnoea and hypoxia over 2 weeks. CXR shows bilateral diffuse ground-glass opacities. CT shows extensive ground-glass changes with a crazy-paving pattern. BAL shows PAS-positive, foamy material. What is the diagnosis?

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Correct answer: E — Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) is rare in children and can be caused by GM-CSF autoantibodies, genetic mutations (CSF2RA/B, SFTPB, SFTPC, ABCA3, NKX2-1), or secondary to immunodeficiency. BAL shows milky fluid with PAS-positive lipoproteinaceous material. Treatment includes whole lung lavage and GM-CSF therapy.

Reference: RCH Melbourne – 2024 – Clinical Practice Guidelines: Interstitial Lung Disease in Children