At baseline, 60% of patients with CANDLE and all patients with SAVI had detectable autoantibodies against endothelial antigens and targets including phospholipids (lupus anticoagulant and anti-cardiolipin Abs), antimyeloperoxidase and proteinase-3, and/or against nuclear antigens (ANA, SSA) and DNA (dsDNA). This evidence concerns the gene PRTN3 and systemic lupus erythematosus.