CTICasesAdult-Onset Cystic Fibrosis
Imaging findings
Chest radiography demonstrates bilateral nodules, bronchial wall thickening, and areas of consolidation, particularly in the right upper lobe. High-resolution CT of the chest reveals extensive tree-in-bud nodularity indicative of infectious bronchiolitis, mild bronchial wall thickening, and surrounding patchy consolidation. Additionally, there is evidence of air trapping, while the pancreas maintains a normal, healthy appearance without the exocrine calcifications or cystic changes typically seen in childhood-onset cases.
Key takeaways
Adult-onset cystic fibrosis is a recognized entity, often presenting in patients in their 30s, 40s, or even older, and is typically associated with non-classic or milder CFTR mutations rather than the common homozygous F508del mutation. These patients frequently present with chronic or recurrent sinopulmonary symptoms, bronchiectasis, or bronchiolitis, but usually lack the classic exocrine pancreatic insufficiency seen in pediatric-onset disease. Despite the name 'cystic fibrosis', the defining pulmonary features are bronchiectasis, airway plugging, and bronchiolitis, rather than true cysts or parenchymal lung fibrosis, which are pancreatic autopsy descriptors.
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