CTICasesCystic Fibrosis with Severe Small Airways Disease
Imaging findings
Chest CT in a young adult with cystic fibrosis demonstrates extensive, diffuse tree-in-bud opacities and centrilobular nodularity involving all lobes, accompanied by mild upper-lobe-predominant bronchiectasis and prominent mosaic perfusion. The diffuse tree-in-bud opacities are chronic, showing no significant change from prior CT scans during periods of clinical stability.
Key takeaways
Although cystic fibrosis is classically characterized by severe bronchiectasis and lobar atelectasis, some patients present with a highly dominant pattern of extensive centrilobular small airways disease and tree-in-bud opacities. This appearance represents chronic, widespread bronchiolitis with inspissated mucus plugs, typically related to chronic colonization with pathogens such as Pseudomonas aeruginosa or Staphylococcus aureus. This presentation must be distinguished from acute infectious exacerbations or other causes of diffuse bronchiolitis, such as excipient lung disease in patients with indwelling catheters.
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