title: Novel diagnostic and therapeutic frontiers in thrombotic anti-phospholipid syndrome.
abstract: The anti-phospholipid syndrome (APS) is an autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity, associated with a persistent positivity for anti-phospholipid antibodies (aPL). The current classification criteria for APS include three laboratory tests: lupus anti-coagulant (LA), anti-cardiolipin (aCL), and anti-β2 glycoprotein-I (β2GPI). To date, the therapeutic approach for thrombotic APS mainly centers on long-term anti-coagulation with a vitamin K antagonist (VKA). APS management may represent a challenge for the treating physicians. Patients with different aPL profiles need a tailored risk-stratified approach. Moreover, in patients with recurrent thrombotic events despite therapy with VKA, or in those with microvascular involvement, new therapeutic options are highly needed. In this review, we aim to elucidate recent findings about new aPL specifities, available risk scoring models, and novel therapeutic approaches in APS management.
authors.affiliation: Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, S. Giovanni Bosco Hospital, Piazza del Donatore di Sangue 3, 10154, Turin, Italy. savino.sciascia@unito.it.|SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy. savino.sciascia@unito.it.
authors.forename: Savino
authors.initials: S
authors.lastname: Sciascia
authors.affiliation: Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, S. Giovanni Bosco Hospital, Piazza del Donatore di Sangue 3, 10154, Turin, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Radin
authors.affiliation: UOSD Hematology and Thrombosis Unit, S. Giovanni Bosco Hospital, Turin, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Bazzan
authors.affiliation: Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases, Coordinating Center of Piemonte and Valle d'Aosta Network for Rare Diseases, S. Giovanni Bosco Hospital, Piazza del Donatore di Sangue 3, 10154, Turin, Italy.|SCDU Nephrology and Dialysis, S. Giovanni Bosco Hospital, Turin, Italy.
authors.forename: Dario
authors.initials: D
authors.lastname: Roccatello
mesh_terms: D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000032:analysis / Q000097:blood; D000911:Antibodies, Monoclonal / Q000494:pharmacology / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000494:pharmacology / Q000627:therapeutic use; D000925:Anticoagulants / Q000494:pharmacology / Q000627:therapeutic use; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D005343:Fibrinolytic Agents / Q000494:pharmacology / Q000627:therapeutic use; D006801:Humans; D006886:Hydroxychloroquine / Q000494:pharmacology / Q000627:therapeutic use; D016682:Lupus Coagulation Inhibitor / Q000032:analysis / Q000097:blood; D011247:Pregnancy; D011248:Pregnancy Complications / Q000175:diagnosis / Q000517:prevention & control / Q000628:therapy; D018570:Risk Assessment / Q000379:methods; D000069552:Rivaroxaban / Q000494:pharmacology / Q000627:therapeutic use; D013927:Thrombosis / Q000175:diagnosis / Q000628:therapy*; D053482:beta 2-Glycoprotein I / Q000032:analysis / Q000097:blood
chemical_list: D017153:Antibodies, Anticardiolipin; D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000925:Anticoagulants; D005343:Fibrinolytic Agents; D016682:Lupus Coagulation Inhibitor; D053482:beta 2-Glycoprotein I; D006886:Hydroxychloroquine; D000069552:Rivaroxaban; C481642:eculizumab
keywords: APS; Pregnancy morbidity; Thrombosis; aPL