A total of 11 patients with a molecularly proven diagnosis of AGS (RNASEH2B, n = 4; ADAR1, n = 2; TREX1, n = 2; IFIH1, n = 2; RNU7-1, n = 1) were treated with a JAK1/2 inhibitor for a median period of 17 (range, 12–48) months (Table 1). The gene discussed is JAK1; the disease is Aicardi-Goutieres syndrome.