CTICasesPulmonary Alveolar Proteinosis Progressing to Fibrosis
Imaging findings
A 55-year-old man with pulmonary alveolar proteinosis diagnosed about 15 years earlier and treated with whole-lung lavage has basal-predominant fibrosis with diffuse symmetric ground glass, dilated bronchi, irregular reticulation, and corkscrew (traction) bronchiectasis in the middle lobe. His PFTs were markedly reduced (FVC 50%, DLCO 30%), and he died within a year despite off-label antifibrotic therapy.
Key takeaways
Pulmonary alveolar proteinosis, usually autoimmune, can uncommonly progress to an IPF-like fibrosis (reported in roughly 20 percent of a case series), a poorly recognized and potentially terminal evolution. A genetic contribution is likely, as surfactant protein C mutations cause protein misfolding, endoplasmic reticulum stress, and type II alveolar epithelial apoptosis that create a profibrotic environment, so genetic sequencing of PAP patients who fibrose would be informative.
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