Diagnosis and management of the antiphospholipid syndrome

Abstract

The antiphospholipid syndrome is a systemic autoimmune disease defined by thrombotic or obstetrical events that occur in patients with persistent antiphospholipid antibodies. Thrombotic antiphospholipid syndrome is characterized by venous, arterial, or microvascular thrombosis. Patients with catastrophic antiphospholipid syndrome present with thrombosis involving multiple organs. Obstetrical antiphospholipid syndrome is characterized by fetal loss after the 10th week of gestation, recurrent early miscarriages, intrauterine growth restriction, or severe preeclampsia.1 The major nonthrombotic manifestations of antiphospholipid-antibody positivity include valvular heart disease, livedo, antiphospholipidantibody-related nephropathy, thrombocytopenia, hemolytic anemia, and cognitive dysfunction. The antiphospholipid syndrome is often associated with other systemic autoimmune diseases such as systemic lupus erythematosus (SLE); however, it commonly occurs without other autoimmune manifestations (primary antiphospholipid syndrome).