DIRA (OMIM 612852) is an autosomal recessive disease caused by missense mutations in the IL1RN gene encoding for the IL-1 receptor antagonist 1; there is a lack of endogenous self-regulation of IL-1 activity, with consequent excessive proinflammatory action of IL-1 itself [146] (Table 1). The gene discussed is IL1B; the disease is sterile multifocal osteomyelitis with periostitis and pustulosis.