CTICasesPulmonary Alveolar Proteinosis Progressing to Fibrosis
Imaging findings
A patient with chronic dyspnea had a scan about 13 years earlier showing a classic geographic crazy-paving pattern of pulmonary alveolar proteinosis. After whole-lung lavage and years of recurrent dyspnea, the current scan shows a coarser fibrotic appearance with reticulation and ground glass, especially subpleural, that has slowly progressed over serial scans.
Key takeaways
Pulmonary alveolar proteinosis, though characteristically producing crazy-paving, can uncommonly progress to lung fibrosis over years, a rare complication of an already rare disease that is documented in a small number of cases. When a PAP patient becomes progressively more symptomatic with a coarsening reticular pattern, fibrotic evolution should be considered.
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