CTICasesCOPA Syndrome
Imaging findings
Chest CT of a 20-year-old female never-smoker with lupus shows progressive, extensive emphysema-like changes (cysts with centrilobular arteries), along with pronounced septal thickening and follicular bronchiolitis. Open lung biopsy showed lymphocytic infiltration and germinal centers.
Key takeaways
COPA syndrome is a rare autosomal dominant autoimmune disorder caused by mutations in the COPA gene. It classically presents in teens or young adults as severe, progressive follicular bronchiolitis, interlobular septal thickening, and cystic/emphysematous lung disease in never-smokers.
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