CTICasesCOPA Syndrome Pulmonary Fibrosis
Imaging findings
A 22-year-old with a family member also affected by the disorder had CT showing markedly advanced fibrosis with extensive cysts and holes, a pattern similar to the more severe surfactant protein C cases shown earlier, with some traction bronchiectasis and an indeterminate nodule; the diagnosis of COPA syndrome was made retrospectively after a causative mutation was identified in the patient's lab.
Key takeaways
COPA syndrome is a familial autoinflammatory disorder that can cause markedly advanced pulmonary fibrosis at a young age, sometimes associated with skeletal features such as pectus carinatum, and like the other familial fibrosing lung diseases discussed, its imaging pattern is difficult to classify using standard idiopathic interstitial pneumonia criteria; recognizing a family history of unexplained fibrosis, particularly at a young age, should prompt genetic evaluation for entities like COPA syndrome even when initially undiagnosed.
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