TREX1 and autoimmune disease: 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.