Of note, heterozygous mutations in TREX1 and the RNASEH2 genes, which cause the type I interferonopathy Aicardi-Goutières syndrome, confer an increased risk for SLE [9, 10] raising the question as to whether constitutive activation of antiviral immunity in parents of patients with SPENCD may also increase the risk for autoimmune disease later in life. Here, TREX1 is linked to systemic lupus erythematosus.