CTICasesConnective tissue disease-associated emphysema mimicking UIP
Imaging findings
CT scan shows extensive centrilobular and paraseptal emphysema in the upper lobes and giant, bubbly subpleural cystic spaces at the lung bases. Despite the basal cystic spaces, there is a straight-edge distribution, and there is an absence of traction bronchiectasis or typical fibrotic walls.
Key takeaways
Giant, thin-walled, subpleural cystic spaces (bubbly lung) at the lung bases can mimic honeycombing but represent atypical emphysema associated with connective tissue disease. The lack of adjacent fibrotic scarring helps differentiate it from UIP.
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