The antiphospholipid syndrome: from pathophysiology to treatment

Abstract

Antiphospholipid antibody syndrome (APS) is an autoimmune acquired thrombophilia characterized by recurrent thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). APS can be primary, if it occurs in the absence of any underlying disease, or secondary, if it is associated with another autoimmune disorder, most commonly systemic lupus erythematosus. The exact pathogenetic mechanism of APS is unknown, but different, not mutually exclusive, models have been proposed to explain how anti-PL autoantibodies might lead to thrombosis and pregnancy morbidity. Diagnosis of APS requires that a patient has both a clinical manifestation (arterial or venous thrombosis and/or pregnancy morbidity) and persistently positive aPL, but the clinical spectrum of the disease encompasses additional manifestations which may affect every organ and cannot be explained exclusively by a prothrombotic state. Treatment for aPL-positive patients is based on the patient's clinical status, presence of an underlying autoimmune disease, and history of thrombotic events. In case of aPL positivity without previous thrombotic events, the treatment is mainly focused on reduction of additional vascular risk factors, while treatment of patients with definite APS is based on long-term anticoagulation. Pregnancy complications are usually managed with low-dose aspirin in association with low molecular weight heparin. Refractory forms of APS could benefit from adding hydroxychloroquine and/or intravenous immunoglobulin to anticoagulation therapy. Promising novel treatments include anti-B cell monoclonal antibodies, new-generation anticoagulants, and complement cascade inhibitors. The objective of this review paper is to summarize the recent literature on APS from pathogenesis to current therapeutic options.

The microbiome in autoimmune diseases

“…© 2018 British Society for Immunology, Clinical and Experimental Immunology, 195:[74][75][76][77][78][79][80][81][82][83][84][85] …”

Antiphospholipid Syndrome: Multiple Manifestations in a Single Patient—A High Suspicion Is Still Needed

“…Control of other cardiovascular risk factors is recommended for all patients [20]. Severe or symptomatic thrombocytopenia and AIHA are treated with steroids in the first-line setting [21]. …”

Annexins in Translational Research: HIDDEN Treasures to Be Found

“…The AnxA5 anticoagulant function might depend on its 4 well-established property to self-assemble on PS-containing membranes into an extensive two-dimensional crystal lattice [42] that hinders the assembly of the procoagulant complexes. In line with such protective function in the blood clotting regulation, anti-AnxA5 autoantibodies are found in patients suffering from antiphospholipid syndrome [43], a disease that manifests clinically as recurrent thrombotic events and is associated with fetal loss [44]. The occurrence of AnxA5 autoantibodies [45] is also linked to autoimmune disorders [46] also observed in some patients suffering from multiple sclerosis or systemic lupus erythematosus.…”