title: 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.
authors.affiliation: Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Viale Benedetto XV, 6, 16132, Genoa, Italy. negrini@unige.it.|Centre of Excellence for Biomedical Research, University of Genoa, Genoa, Italy. negrini@unige.it.
authors.forename: Simone
authors.identifier: http://orcid.org/0000-0003-1267-4320
authors.initials: S
authors.lastname: Negrini
authors.affiliation: Department of Dermatology, University of Genoa, Genoa, Italy.
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Pappalardo
authors.affiliation: Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Viale Benedetto XV, 6, 16132, Genoa, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Murdaca
authors.affiliation: Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Viale Benedetto XV, 6, 16132, Genoa, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Indiveri
authors.affiliation: Department of Internal Medicine, Clinical Immunology Unit, University of Genoa, Viale Benedetto XV, 6, 16132, Genoa, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Puppo
mesh_terms: D000925:Anticoagulants / Q000627:therapeutic use*; D016736:Antiphospholipid Syndrome / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D011247:Pregnancy; D011248:Pregnancy Complications / Q000188:drug therapy* / Q000453:epidemiology / Q000503:physiopathology*; D012737:Sex Factors
chemical_list: D000925:Anticoagulants
keywords: Anti-β2 glycoprotein-1 antibody; Anticardiolipin antibody; Antiphospholipid syndrome; Lupus anticoagulant; Pregnancy morbidity; Thrombosis