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Lymphangioleiomyomatosis

Imaging findings

Serial CT chest scans over ten years show progression from discrete, thin-walled, round cysts distributed throughout both lungs into confluent, destructive, emphysema-like spaces with loss of pulmonary vasculature.

Key takeaways

Lymphangioleiomyomatosis (LAM) is a low-grade neoplastic disease of women, characterized by diffuse cystic lung destruction. Cysts can merge and mimic panlobular emphysema on CT. Proposed mechanisms include matrix metalloproteinase-mediated parenchymal destruction rather than simple airway obstruction.

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