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Pulmonary Alveolar Proteinosis

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Imaging findings

Chest radiograph shows reduced lung volumes and diffuse, patchy bilateral opacities. Chest CT demonstrates a classic "crazy-paving" pattern characterized by ground-glass opacities superimposed on thickened interlobular and intralobular septa, presenting in a heterogeneous and patchy distribution predominantly in the upper lobes.

Key takeaways

Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by the accumulation of surfactant-like lipoproteinaceous material within the alveoli. The classic high-resolution CT hallmark of PAP is the "crazy-paving" pattern (thickened septa on a background of ground-glass opacity), which can sometimes present in a patchy, heterogeneous distribution rather than diffuse involvement. Although PAP typically affects younger adults, it can occasionally present in older patients, where it can be misdiagnosed as atypical pneumonia, pulmonary hemorrhage, or mucinous adenocarcinoma.

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