Cutting-edge issues in coronary disease and the primary antiphospholipid syndrome

Antiphospholipid syndrome (APS) is the most frequent cause of venous and arterial thrombotic events in young patients. The brain arterial tree is primarily affected, but coronary ischemic manifestations are also relatively frequent. Coronary involvement was suggested to be closely related to the accelerated atherosclerosis linked to the underlying disease in APS associated to systemic autoimmune diseases, in particular, systemic lupus erythematosus. However, arterial ischemic events can occur in primary APS--with no other systemic disorders--even in the absence of traditional cardiovascular risk factors and overt atherosclerosis. From a biological point of view, this finding speaks in favor for a pro-coagulant activity of anti-phospholipid antibodies rather than for their role in atherosclerotic plaque formation. On the other hand, the clinical challenge is to avoid the risk to misdiagnose young patients with potentially life-threatening symptoms, such as myocardial infarction (MI). In fact, the occurrence of nonspecific symptoms related to coronary ischemic events is frequently misdiagnosed because of its rarity in young patients. This issue is well illuminated by two cases of MI in young patients reported in the manuscript together with a systematic review of the associations and implications of coronary ischemic events in APS.