Antiphospholipid syndrome (APS) refers to an autoimmune disease characterized by multiple arterial or venous thrombosis, pregnancy complications, and/or thrombocytopenia with positivity for antiphospholipid antibodies (aPL), including lupus anticoagulants (LA), anticardiolipin antibodies (aCL), and anti-β2 glycoprotein-I (aβ2GPI) (1–4). Here, ACLY is linked to autoimmune polyendocrinopathy.