IFNA1 and autoimmune interstitial lung disease-arthritis syndrome: While confirming the core organ features (lung, joint and kidney) of COPA syndrome, our data expand the phenotype to include cardiac, skin and digestive features, further demonstrating the clinical overlap with SAVI and other type I interferonopathies.In view of current (JAK inhibitors) and potential future targeted therapies, we suggest a requirement to assess IFN pathway status and/or perform sequencing in the case of suggestive features, even in the absence of a familial history.