APS is characterized by the activation of TLR1 [175], TLR2 [175,176,177], TLR4 [170,176,178,179], TLR6 [175], TLR7 [180,181,182], TLR8 [181,182,183], NOD2 [184] and NLRP3 [185]. Here, TLR4 is linked to autoimmune polyendocrinopathy.