Mice lacking A20 specifically in CD11c+ dendritic cells show phenotypical similarities to human SLE including glomerulonephritis, presence of antibodies against dsDNA, ribonucleoprotein and cardiolipin, thrombocytopenia and autoimmune lymphoproliferative syndrome (ALPS) including enhanced spontaneous lymphocyte activation, accumulation of double negative lymphocytes and myeloproliferative syndrome (Kool et al., 2011). Here, TNFAIP3 is linked to autoimmune lymphoproliferative syndrome.