With our study, we further characterized the group of patients with normal complement values and proposed a disease model encompassing various degrees of autoinflammatory and autoimmune pathogenesis, ranging from the typical form of SLE, characterized by prominent autoimmune features, hypocomplementemia and high clinical severity, and a subset of disease with high IFN inflammation, lower autoimmunity and, at least in our experience, milder severity. Here, IFNA1 is linked to systemic lupus erythematosus.