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J Autoimmun. 2014 Feb-Mar;48-49:20-5. doi: 10.1016/j.jaut.2014.01.006. Epub 2014 Jan 24.

Diagnosis and classification of the antiphospholipid syndrome.

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Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain; Division of Rheumatology, Immunology and Allergy, Section of Clinical Sciences, Brigham and Women's Hospital, Boston, MA, USA.
Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain. Electronic address:


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). Some estimates indicate that the incidence of the APS is around 5 new cases per 100,000 persons per year and the prevalence around 40-50 cases per 100,000 persons. The aPL are positive in approximately 13% of patients with stroke, 11% with myocardial infarction, 9.5% of patients with deep vein thrombosis and 6% of patients with pregnancy morbidity. The original classification criteria for the APS were formulated at a workshop in Sapporo, Japan, in 1998, during the 8th International Congress on aPL. The Sapporo criteria, as they are often called, were revised at another workshop in Sydney, Australia, in 2004, during the 11th International Congress on aPL. At least one clinical (vascular thrombosis or pregnancy morbidity) and one laboratory (anticardiolipin antibodies, lupus anticoagulant or anti-β2-glycoprotein I antibodies) criterion had to be met for the classification of APS.


Anticardiolipin antibodies; Antiphospholipid syndrome; Catastrophic antiphospholipid syndrome; Lupus anticoagulant

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