This case shows the need for caution and further assessment if IgA anti-β2GPI is the only positive aPL on enzyme-linked immunosorbent assay (ELISA).[6] According to the revised Sapporo classification criteria of the APS in 2006,[7] the laboratory criteria of APS require positive LA, IgM, IgG aCL, IgM, or IgG anti-β2GPI twice in an interval of at least 12 weeks. The gene discussed is CD40LG; the disease is autoimmune polyendocrinopathy.