Other outstanding cases are somatic variants in NOD2 causing Blau syndrome [52]; NLRC4 causing NOMID (neonatal-onset multisystem inflammatory disease) [53]; and UBA1 associated with the late-onset VEXAS (vacuole, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, a severe X-linked disorder affecting males and restricted to cells of the myeloid line [54]. The gene discussed is UBA1; the disease is CINCA syndrome.