CTICasesPulmonary Hemosiderosis with Centroacinar Emphysema
Imaging findings
Chest radiograph reveals symmetric upper lobe volume loss and superior elevation of both hila. High-resolution CT shows a bizarre pattern of upper-lobe-predominant cystic air spaces and centriacinar emphysema radiating from the hilum, accompanied by mild traction bronchiolectasis and faint basilar ground-glass opacities, with no significant interstitial fibrosis. Surgical lung biopsy demonstrates centroacinar emphysema, extensive alveolar hemosiderosis, iron-encrusted elastic fibers in vessel walls, and occasional polarizable particles suggestive of silica.
Key takeaways
Conspicuous upper-lobe-predominant centrilobular emphysema and volume loss in a lifelong non-smoker is highly unusual and warrants a detailed search for inhalational or occupational exposures. The pathologic finding of prominent hemosiderin deposition (hemosiderosis) and iron-encrusted elastic fibers in the vessel walls suggests a diagnosis of idiopathic pulmonary hemosiderosis or chronic occult alveolar hemorrhage, which can lead to bizarre cystic lung destruction and mimic severe emphysema. When faced with atypical emphysema patterns, clinicians should investigate potential exposures to mineral dusts, silica, metal fumes, or fiberglass that can cause localized alveolar injury and secondary hemorrhage.
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