Most autoinflammatory disorders present in childhood and neonatal onset is predominantly observed in patients with: the sterile osteomyelitis syndrome, DIRA; the most severe form of the cryopyrin associated periodic syndromes (CAPS), NOMID/CINCA; and the Type I interferonopathies, such as AGS, SAVI and USP18 deficiency. Here, USP18 is linked to Aicardi-Goutieres syndrome.