CTICasesCystic Lung Disease Mimicking UIP — Possible Airspace Enlargement with Fibrosis (AEF)
Imaging findings
CT in a patient with a clinical phenotype of IPF (60s, male, former smoker, clubbing, crackles) demonstrates basal-predominant cystic or hole-like spaces without significant reticulation. The airways taper normally and pass through the cystic areas without typical traction bronchiectasis. Upper lobes show emphysematous changes from smoking. The coronal view shows no significant volume loss despite the extensive cystic change. The outside institution diagnosed IPF. The consulting radiologist notes the lack of reticulation, absence of volume loss, and normal-caliber tapering airways as features that do not fit typical UIP. A reference is provided on airspace enlargement with fibrosis (AEF) from Tom Colby and group.
Key takeaways
Smokers can develop an entity distinct from UIP/IPF in which emphysematous airspace enlargement occurs with surrounding fibrosis, producing large holes that mimic honeycomb cysts. Differentiating features from true UIP honeycomb include: absence of significant reticulation, lack of volume loss, normal-caliber tapering airways passing through the cystic areas rather than traction bronchiectasis, and upper lobe emphysema. This entity has been described as airspace enlargement with fibrosis (AEF) and has a different natural history from UIP/IPF. Recognizing this distinction avoids incorrect prognostication and inappropriate antifibrotic therapy.
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