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. The gene discussed is USP18; the disease is CINCA syndrome.