A diagnosis of APS is based on the revised Sapporo criteria and requires the presence of at least one clinical criteria (vascular thrombosis and/or pregnancy morbidity) and one laboratory criteria (persistence of at least 12 weeks of lupus anticoagulant and/or medium-high titers of IgG or IgM autoantibodies against β2GPI or cardiolipin) [8] (Table 1). The gene discussed is CD40LG; the disease is autoimmune polyendocrinopathy.