Systemic Lupus Erythematosus and Antiphospholipid Syndrome

Abstract

The antiphospholipid syndrome (APS) is defined by the occurrence of venous and arterial thromboses, often multiple, and recurrent fetal losses, frequently accompanied by a moderate thrombocytopenia, in the presence of antiphospholipid antibodies (aPL), namely, lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or anti-β2-glycoprotein I (β2GPI) antibodies (Gómez-Puerta and Cervera, J Autoimmun 48–49:20–25, 2014). The APS can be found in patients having neither clinical nor laboratory evidence of another definable condition (primary APS), or it may be associated with other diseases, mainly systemic lupus erythematosus (SLE), but occasionally with other autoimmune conditions, infections, drugs, and malignancies. Primary APS patients rarely progress to SLE. Only 8 % of 128 patients, who were followed up for about 9 years, developed lupus, and a positive Coombs’ test was a clinically significant predictor of progression. The aPL can appear in different scenarios, such as asymptomatic “carrier” patients for aPL, “classical” APS with recurrent venous and/or arterial thrombosis, APS affecting otherwise healthy women with recurrent pregnancy loss, patients with aPL positivity with non-thrombotic aPL manifestations (i.e., thrombocytopenia, hemolytic anemia, or livedo reticularis), or, in a small subset of patients, as a life-threatening form characterized by a rapid development of microthrombosis that led to rapid multiorgan failure, which is termed catastrophic APS.