Some gene variants were first reported in association with other diseases with an inflammatory component (e.g., Aicardi-Goutières syndrome (AGS), stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI), Noonan syndrome, etc.)and linked with SLE later [8–11, 15] (Table 1 summarizes monogenic jSLE causes reported since 2020). This evidence concerns the gene STING1 and Aicardi-Goutieres syndrome.