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. Here, IFNA1 is linked to systemic lupus erythematosus.