Mutations of the TREX1 gene are a hallmark of Aicardi–Goutières syndrome (AGS) (Crow et al., 2015), characterized by a lupus reminiscent picture with high IFN-α in peripheral blood and CSF, white matter lesions, autoantibody positivity and cytopenia (Ramantani et al., 2010; Crow and Manel, 2015; Cuadrado et al., 2015; Cattalini et al., 2016). This evidence concerns the gene TREX1 and Aicardi-Goutières syndrome.