IFNA1 and Aicardi-Goutieres syndrome: Aicardi-Goutières syndrome (AGS) is a monogenic autoinflammatory disorder that overlaps phenotypically with congenital viral infection and systemic lupus erythematosus (SLE), and is characterized by constitutive upregulation of type I interferon (IFN) in the serum and cerebrospinal fluid1.