Abstract
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 prevalence is higher among patients with systemic lupus erythematosus (around 30%), deep vein thrombosis (30%), stroke in younger than 50 year-old (25%), and recurrent fetal losses (10%).
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.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Preview
Unable to display preview. Download preview PDF.
References
Asherson RA, Cervera R, Piette JC, Shoenfeld Y (eds.). The Antiphospholipid Syndrome II – Autoimmune Thrombosis. Amsterdam, Elsevier; 2002.
Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol 1992; 19: 508–12.
Piette JC, Cervera R, Levy RA, Nasonov EL, Triplett DA, Shoenfeld Y. The catastrophic antiphospholipid syndrome – Asherson’s syndrome. Ann Med Interne 2003; 154: 195–6.
Asherson RA, Pierangeli SS, Cervera R. Is there a microangiopathic antiphospholipid syndrome? Ann Rheum Dis 2007; 66: 429–32.
Petri M. Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun 2000; 15: 145–51.
Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, et al. Antiphospholipid syndrome. Clinical and immunologic manifestations and patterns of disease expression in a cohort of 1000 patients. Arthritis Rheum 2002; 46: 1019–27.
Bucciarelli S, Espinosa G, Cervera R, Erkan D, Gómez-Puerta JA, Ramos-Casals M, et al. Mortality in the catastrophic antiphospholipid syndrome. Causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum 2006; 54: 2568–76.
Hughes GRV. Thrombosis, abortion, cerebral disease and the lupus anticoagulant. Br Med J 1983; 287: 1088–9.
Espinosa G, Cervera R, Font J, Shoenfeld Y. Antiphospholipid syndrome: Pathogenic mechanisms. Autoimmun Rev 2003; 2: 86–93.
Brandt JT, Triplett DA, Alving B, Scharrer I. Criteria for the diagnosis of lupus anticoagulants: An update. Thromb Haemost 1995; 74: 1185–90.
Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RHWM, De Groot PG, Koike T, Meroni PL, Reber G, Shoenfeld Y, Tincani A, Vlachoyiannopoulos PG, Krilis SA. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4: 295–306.
Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, Khamashta MA, Shoenfeld Y for the Catastrophic Antiphospholipid Syndrome Registry Project Group. Catastrophic antiphosphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12: 530–4.
Kaul M, Erkan D, Sammaritano L, Lockshin MD. Assessment of the 2006 revised antiphospholipid syndrome classification criteria. Ann Rheum Dis 2007; 66: 927–30.
Cervera R, Font J, Gómez-Puerta JA, Espinosa G, Cucho M, Bucciarelli S, Ramos-Casals, M, Ingelmo M, Piette JC, Shoenfeld Y, RA Asherson for the Catastrophic Antiphospholipid Syndrome Registry Project Group. Validation of the preliminary criteria for the classification of catastrophic antiphospholipid syndrome. Ann Rheum Dis 2005; 64: 1205–9.
Lim W, Crowther MA, Eikelboom JW. Management of antiphospholipid antibody syndrome: A systematic review. JAMA 2006; 295: 1050–7.
Alarcón-Segovia D, Boffa MC, Branch W, Cervera R, Gharavi A, Khamashta M, Shoenfeld Y, Wilson W, Roubey R. Prophylaxis of the antiphospholipid syndrome: A consensus report. Lupus 2003; 12: 499–503.
Erkan D, Harrison MJ, Levy R, Peterson M, Petri M, Sammaritano L, Unalp-Arida A, Vilela V, Yazici Y, Lockshin MD. Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: A randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibody-positive individuals. Arthritis Rheum 2007; 56: 2382–91.
Tincani A, Branch W, Levy RA, Piette JC, Carp H, Rai RS, Khamashta M, Shoenfeld Y. Treatment of pregnant patients with antiphospholipid syndrome. Lupus 2003; 12: 524–9.
Carmona F, Font J, Azulay M, Creus M, Fàbregues F, Cervera R, Puerto B, Balasch J. Risk factors associated with fetal losses in treated antiphospholipid syndrome pregnancies: A multivariate analysis. Am J Reprod Immunol 2001; 46: 274–279.
Cervera R, Balasch J. The management of pregnant patients with antiphospholipid syndrome. Lupus 2004; 13: 683–7.
Author information
Authors and Affiliations
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2008 Humana Press, a part of Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Cervera, R., Asherson, R.A. (2008). Antiphospholipid Syndrome. In: Shoenfeld, Y., Cervera, R., Gershwin, M.E. (eds) Diagnostic Criteria in Autoimmune Diseases. Humana Press. https://doi.org/10.1007/978-1-60327-285-8_2
Download citation
DOI: https://doi.org/10.1007/978-1-60327-285-8_2
Publisher Name: Humana Press
Print ISBN: 978-1-60327-427-2
Online ISBN: 978-1-60327-285-8
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)