Since JAK1 and TYK2 play a pivotal role in IFNAR1 and IFNAR2 activation and in type I IFNs response, JAKi are considered promising in reducing the inflammation caused by the IFNs overproduction (Tonduti, et al., 2020; Vanderver et al., 2020); current ongoing clinical trials are exploring the use of baricitinib (a JAK1/JAK2 non selective inhibitor) in AGS and AGS-related interferonopathies (NCT03921554, NCT04517253, clinicaltrials.gov). Here, JAK2 is linked to Aicardi-Goutieres syndrome.