title: Antiphospholipid syndrome: frequency, main causes and risk factors of mortality.
abstract: The presence of antiphospholipid antibodies has been shown to be related to an increased risk of thrombotic events. In patients with definite antiphospholipid syndrome (APS), that is, those who have had thrombosis and at least two positive determinations of antiphospholipid antibodies, secondary thromboprophylaxis with long-term anticoagulation therapy results in a low rate of recurrent thrombotic events, ranging from 0.016 to 0.031 events per patient per year. Thrombotic complications are, however, the most common cause of death in APS. The mortality rate in a large European cohort of patients with APS during a 5-year study period was 5.3%, and up to 40% of the deaths in this cohort were attributed to severe thrombotic events such as myocardial infarction, stroke and pulmonary embolism. Catastrophic APS is an unusual form of the disease, being observed in less than 1% of reported cases of APS, which is associated with a much higher mortality rate than classical APS. The combined use of anticoagulation, corticosteroids, plasma exchange and intravenous immunoglobulin therapy could result in a dramatic reduction in mortality, by approximately 20%, in patients with catastrophic APS.
authors.affiliation: Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Villarroel, Barcelona, Catalonia, Spain.
authors.forename: Gerard
authors.initials: G
authors.lastname: Espinosa
authors.forename: Ricard
authors.initials: R
authors.lastname: Cervera
mesh_terms: D017152:Antibodies, Antiphospholipid; D016736:Antiphospholipid Syndrome / Q000453:epidemiology* / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D006801:Humans; D012307:Risk Factors
chemical_list: D017152:Antibodies, Antiphospholipid
references.citation: J Rheumatol. 2004 Aug;31(8):1560-7
references.pmid: 15290736
references.citation: Arthritis Rheum. 2006 Aug;54(8):2568-76
references.pmid: 16868979
references.citation: Ann Intern Med. 1992 Aug 15;117(4):303-8
references.pmid: 1637025
references.citation: Semin Thromb Hemost. 2008 Apr;34(3):290-4
references.pmid: 18720310
references.citation: Medicine (Baltimore). 1998 May;77(3):195-207
references.pmid: 9653431
references.citation: Arthritis Rheum. 2007 Dec 15;57(8):1487-95
references.pmid: 18050167
references.citation: Am J Med. 1998 Apr;104(4):332-8
references.pmid: 9576405
references.citation: Am J Med. 1998 Apr;104(4):381-5
references.pmid: 9576413
references.citation: Arch Intern Med. 2002 May 27;162(10):1164-9
references.pmid: 12020188
references.citation: Medicine (Baltimore). 2001 Nov;80(6):355-77
references.pmid: 11704713
references.citation: Ann Rheum Dis. 2009 Sep;68(9):1428-32
references.pmid: 18801761
references.citation: J Rheumatol. 2007 May;34(5):923-6
references.pmid: 17477476
references.citation: J Rheumatol. 2000 Dec;27(12):2817-21
references.pmid: 11128669
references.citation: J Thromb Haemost. 2006 Feb;4(2):295-306
references.pmid: 16420554
references.citation: Lupus. 2003;12(7):530-4
references.pmid: 12892393
references.citation: Arthritis Rheum. 2002 Apr;46(4):1019-27
references.pmid: 11953980
references.citation: Thromb Haemost. 2005 Apr;93(4):694-9
references.pmid: 15841314
references.citation: Lupus. 2009 Sep;18(10):905-12
references.pmid: 19671791
references.citation: Ann N Y Acad Sci. 2007 Jun;1108:448-56
references.pmid: 17894009
references.citation: J Rheumatol. 2007 Feb;34(2):346-52
references.pmid: 17304658
references.citation: N Engl J Med. 1995 Apr 13;332(15):993-7
references.pmid: 7885428
references.citation: J Autoimmun. 2009 Feb;32(1):1-6
references.pmid: 19059760
references.citation: J Rheumatol. 1992 Apr;19(4):508-12
references.pmid: 1593568