AGS presents with a phenotypic overlap of early-onset encephalopathy, mimicking congenital viral infection (cerebrospinal fluid lymphocytosis and elevated IFN-α levels, loss of white matter, and basal ganglia calcifications), and features of systemic lupus erythematosus (SLE), including cytopenias, oral ulcers, arthritis, perniosis with cutaneous erythematous lesions, and autoantibodies [4, 5]. Here, IFNA1 is linked to Aicardi-Goutieres syndrome.