The high-risk aPL profile as defined by the EULAR recommendations for the management of APS in adults includes at least one of the following: (i) the presence of LAC (measured according to International Society for Thrombosis and Haemostasis guidelines) [5]; (ii) double (any combination of LAC, aCL or aß2GPI antibodies) or triple aPL positivity; and (iii) the presence of persistently high aPL titres [19]. The gene discussed is ACLY; the disease is autoimmune polyendocrinopathy.