CTICasesCOPA syndrome
Imaging findings
Chest CT scans in young patients show diffuse centrilobular nodules representing follicular bronchiolitis, progressive pulmonary fibrosis, pulmonary hemorrhage, and thin-walled cysts, associated with inflammatory arthritis.
Key takeaways
COPA syndrome is a rare genetic autoimmune disorder caused by mutations in the COPA gene. It presents with a combination of follicular bronchiolitis, progressive pulmonary fibrosis, pulmonary hemorrhage, and inflammatory arthritis, often misdiagnosed as juvenile rheumatoid arthritis.
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See all cases from August 31, 2018 →