CTICasesAlpha-1 Antitrypsin Deficiency with Prominent Bronchiectasis
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Imaging findings
This patient's imaging demonstrates bilateral bronchiectasis, mosaic attenuation, and bronchial thickening. The bronchiectasis is predominantly cylindrical and cystic, with hilar-predominant peribronchial thickening, and some areas of developing emphysema. Concurrent evidence of cirrhosis and portal hypertension is also noted.
Key takeaways
Alpha-1 antitrypsin (AAT) deficiency is a genetic disorder typically associated with panlobular emphysema, but can also manifest with prominent bronchiectasis, sometimes preceding or dominating the emphysematous changes. The condition also commonly causes liver disease, leading to cirrhosis and portal hypertension, highlighting its multi-organ impact.
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