Interleukin-1–Receptor Antagonist in the Muckle–Wells Syndrome

Abstract

To the Editor: Studies of hereditary inflammatory disorders have identified novel genes and pathways that may be involved in inflammation and apoptosis generally. Mutations in one such gene, variously named NALP3, CIAS1, and PYPAF1, were recently identified as the cause of the Muckle–Wells syndrome and the familial cold autoinflammatory syndrome¹ and have lately also been associated with neonatal-onset multisystem inflammatory disease.² Interleukin-1 is a key proinflammatory cytokine that contributes to increased synthesis of serum amyloid A protein by hepatocytes during the acute-phase response. The availability of a recombinant interleukin-1–receptor antagonist for clinical use enabled us to undertake a trial of . . .