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Pulmonary Alveolar Microlithiasis

Imaging findings

CT chest shows bilateral, lower-lobe predominant, perilymphatic-like interstitial thickening and subpleural lines without dense calcifications. Pathology shows intra-alveolar lamellar calcified bodies (microliths) and thickened alveolar walls.

Key takeaways

Pulmonary alveolar microlithiasis (PAM) is a rare autosomal recessive disease characterized by intra-alveolar calcium phosphate deposition. While classically presenting with dense 'sand storm' calcifications, atypical or early cases can present with non-calcified interstitial thickening mimicking other ILDs.

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