Results: The cohort included 8 patients with SAVI (STING1/TMEM173 mutation), 7 patients with AGS (Aicardi-Goutières syndrome), 3 patients with SPENCD (spondyloenchondrodysplasia), 3 patients with COPA syndrome, 2 patients with DNAseII deficiency, 2 patients with RNAseTII mutation, 1 patient with PRAAS/Candle syndrome, and 3 patients with presumed monogenic type I interferonopathy. Here, STING1 is linked to Aicardi-Goutieres syndrome.