CTICasesChronic Pulmonary Alveolar Proteinosis with Atypical and Fibrotic Patterns
Imaging findings
A young woman with years of dyspnea has profound interlobular septal thickening with mild apical reticulation and fibrosis, more suggestive of a deposition disease (like Niemann-Pick) than typical crazy paving, but surgical biopsy showed chronic alveolar proteinosis depositing in the lymphatics. A second, older woman shows ground glass and consolidation with areas of traction and fibrosis and mild mosaic attenuation that progressed over three years, again without a classic crazy-paving pattern.
Key takeaways
Chronic pulmonary alveolar proteinosis can deviate from the classic geographic crazy-paving appearance, instead showing profound interlobular septal thickening that mimics a deposition disease, or evolving into traction fibrosis over years. These atypical and fibrotic presentations broaden the imaging spectrum of PAP, and superimposed mycobacterial or nocardial infection can further complicate the appearance.
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