CTICasesLymphangioleiomyomatosis (LAM) Mimicking Alpha-1 Antitrypsin Deficiency
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Imaging findings
Chest radiograph and CT showed widespread cystic changes, initially suggestive of panlobular emphysema, particularly worse at the bases. However, closer inspection of the CT revealed relatively large, stretched vessels within the cystic areas, unlike the typically spindly vessels seen in severe alpha-1 antitrypsin deficiency emphysema. The cystic holes were also more homogeneous from top to bottom, lacking the clear apicobasal gradient seen in alpha-1 antitrypsin deficiency.
Key takeaways
This case highlights how LAM can mimic alpha-1 antitrypsin deficiency on initial imaging. A key distinguishing feature is the vascularity: in LAM, normal lung tissue is often interspersed between cysts, or compressed around them, maintaining some capillary bed, thus vessels may appear larger than expected for the amount of destroyed lung. In contrast, severe panlobular emphysema in alpha-1 antitrypsin deficiency typically shows very spindly vessels and confluent destruction with no normal lung tissue, giving a 'big void' appearance.
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