title: Immunotherapy in antiphospholipid syndrome.
abstract: Antiphospholipid syndrome (APS) is a disorder characterized by the association of arterial or venous thrombosis and/or pregnancy morbidity with the presence of antiphospholipid antibodies (anticardiolipin antibodies, lupus anticoagulant antibodies, and/or anti-β2-glycoprotein I antibodies). Thrombosis is the major manifestation in patients with aPLs, but the spectrum of symptoms and signs associated with aPLs has broadened considerably, and other manifestations, such as thrombocytopenia, non-thrombotic neurological syndromes, psychiatric manifestations, livedo reticularis, skin ulcers, hemolytic anemia, pulmonary hypertension, cardiac valve abnormality, and atherosclerosis, have also been related to the presence of those antibodies. Several studies have contributed to uncovering the basis of antiphospholipid antibody pathogenicity, including the targeted cellular components, affected systems, involved receptors, intracellular pathways used, and the effector molecules that are altered in the process. Therapy for thrombosis traditionally has been based on long-term oral anticoagulation; however, bleeding complications and recurrence despite high-intensity anticoagulation can occur. The currently accepted first-line treatment for obstetric APS (OAPS) is low-dose aspirin plus prophylactic unfractionated or low-molecular-weight heparin (LMWH). However, in approximately 20% of OAPS cases, the final endpoint, i.e. a live birth, cannot be achieved. Based on all the data obtained in different research studies, new potential therapeutic approaches have been proposed, including the use of new oral anticoagulants, statins, hydroxychloroquine, coenzyme Q10, B-cell depletion, platelet and TF inhibitors, peptide therapy or complement inhibition among others. Current best practice in use of these treatments is discussed.
authors.affiliation: Maimonides Institute for Research in Biomedicine of Cordoba (IMIBIC), Reina Sofia University Hospital, University of Cordoba, Córdoba, Spain. Electronic address: rosario.lopez.exts@juntadeandalucia.es.
authors.forename: Ch
authors.initials: Ch
authors.lastname: Lopez-Pedrera
authors.affiliation: Maimonides Institute for Research in Biomedicine of Cordoba (IMIBIC), Reina Sofia University Hospital, University of Cordoba, Córdoba, Spain.
authors.forename: M A
authors.initials: MA
authors.lastname: Aguirre
authors.affiliation: Maimonides Institute for Research in Biomedicine of Cordoba (IMIBIC), Reina Sofia University Hospital, University of Cordoba, Córdoba, Spain.
authors.forename: P
authors.initials: P
authors.lastname: Ruiz-Limon
authors.affiliation: Maimonides Institute for Research in Biomedicine of Cordoba (IMIBIC), Reina Sofia University Hospital, University of Cordoba, Córdoba, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Pérez-Sánchez
authors.affiliation: Maimonides Institute for Research in Biomedicine of Cordoba (IMIBIC), Reina Sofia University Hospital, University of Cordoba, Córdoba, Spain.
authors.forename: Y
authors.initials: Y
authors.lastname: Jimenez-Gomez
authors.affiliation: Maimonides Institute for Research in Biomedicine of Cordoba (IMIBIC), Reina Sofia University Hospital, University of Cordoba, Córdoba, Spain.
authors.forename: N
authors.initials: N
authors.lastname: Barbarroja
authors.affiliation: Lupus Research Unit, St. Thomas Hospital, London, UK.
authors.forename: M J
authors.initials: MJ
authors.lastname: Cuadrado
mesh_terms: D000818:Animals; D000925:Anticoagulants / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000276:immunology / Q000378:metabolism / Q000628:therapy*; D001688:Biological Products / Q000627:therapeutic use; D006801:Humans; D006886:Hydroxychloroquine / Q000627:therapeutic use; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors / Q000627:therapeutic use; D007167:Immunotherapy; D000069283:Rituximab / Q000627:therapeutic use; D013925:Thromboplastin / Q000037:antagonists & inhibitors; D014451:Ubiquinone / Q000031:analogs & derivatives / Q000627:therapeutic use
chemical_list: D000925:Anticoagulants; D001688:Biological Products; D019161:Hydroxymethylglutaryl-CoA Reductase Inhibitors; D014451:Ubiquinone; D000069283:Rituximab; D006886:Hydroxychloroquine; D013925:Thromboplastin; C024989:coenzyme Q10
keywords: Antiphospholipid syndrome; New therapeutic approaches; Pathogenesis