CTICasesDiffuse Pulmonary Amyloidosis with Rapidly Progressive Basal and Peripheral Emphysema
Imaging findings
Sequential chest radiographs and CT scans demonstrate rapidly progressive, severe emphysema predominantly involving the lung bases and peripheral subpleural regions. This is accompanied by ground-glass opacities representing a combination of chronic edema and hypoventilation in crowded, remaining lung parenchyma. Transbronchial and open lung biopsies confirmed light-chain lambda pulmonary amyloidosis associated with multiple myeloma.
Key takeaways
Pulmonary amyloidosis or light-chain deposition disease can rarely present with progressive, severe, basal-predominant emphysema and cystic changes instead of typical nodules or diffuse interstitial thickening. The proposed mechanism is elasto-lysis and tissue destruction driven by an intense inflammatory response, where macrophages attempting to digest the amyloid deposits release elastases and other digestive enzymes that destroy the alveolar elastic tissue.
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