Neonatal-onset multisystem inflammatory disease responsive to interleukin-1beta inhibition

Eighteen patients with neonatal-onset multisystem inflammatory disease are selected to receive anakinra, an interleukin-1-receptor antagonist (1 to 2 mg per kilogram of body weight per day subcutaneously). Daily injection of anakinra markedly improved clinical and laboratory manifestation in patients with neonatal-onset multisystem inflammatory disease, with or without cold-induced autoinflammatory syndrome 1 mutations.