Antiphospholipid Syndrome: Frequency, Main Causes and Risk Factors of Mortality

Overview

Journal Nat Rev Rheumatol

Specialty Rheumatology

Date 2010 Apr 14

PMID 20386563

Citations 25

Authors

Gerard Espinosa

Ricard Cervera

Affiliations

Soon will be listed here.

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.

Citing Articles

Antiphospholipid syndrome in patients with fetal death: a prospective longitudinal cohort study.

Xie A, Jin Z, Li C, Li C, Luo G, Zhang X Clin Exp Med. 2025; 25(1):78.

PMID: 40063133 PMC: 11893624. DOI: 10.1007/s10238-025-01607-0.

Long-Term Follow-Up of Patients With Positive Antiphospholipid Antibodies After Fetal Death: Five Typical Cases From a Prospective Cohort Study.

Xie A, Liu Z, Wang S, Yuan M, Xie L, Liu S Immun Inflamm Dis. 2025; 13(2):e70158.

PMID: 39945244 PMC: 11822662. DOI: 10.1002/iid3.70158.

Clinical and Serological Findings of Patients with Antiphospholipid Syndrome in Mashhad, Iran, from 2008 to 2018: A Retrospective, Descriptive, Cross-sectional Study.

Rezaieyazdi Z, Sahebari M, Shahideh K, Joghatayi M, Khodashahi M Eur J Rheumatol. 2024; 11(4):390-398.

PMID: 39651806 PMC: 11639594. DOI: 10.5152/eurjrheum.2024.23019.

Platelets and the Lectin Pathway of Complement Activation in Patients with Systemic Lupus Erythematosus or Antiphospholipid Syndrome.

Vils S, Troldborg A, Hvas A, Thiel S TH Open. 2023; 7(2):e155-e167.

PMID: 37333022 PMC: 10270747. DOI: 10.1055/a-2087-0314.

Evaluation of a gene signature related to thrombotic manifestations in antiphospholipid syndrome.

Cardoso Jacintho B, Mazetto Fonseca B, Hounkpe B, Oliveira J, Dos Santos A, de Oliveira Vaz C Front Med (Lausanne). 2023; 10:1139906.

PMID: 37035297 PMC: 10076702. DOI: 10.3389/fmed.2023.1139906.

References

Giron-Gonzalez J, Garcia del Rio E, Rodriguez C, Rodriguez-Martorell J, Serrano A . Antiphospholipid syndrome and asymptomatic carriers of antiphospholipid antibody: prospective analysis of 404 individuals. J Rheumatol. 2004; 31(8):1560-7. View

Bucciarelli S, Espinosa G, Cervera R, Erkan D, Gomez-Puerta J, Ramos-Casals M . Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 2006; 54(8):2568-76. DOI: 10.1002/art.22018. View

Rosove M, Brewer P . Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients. Ann Intern Med. 1992; 117(4):303-8. DOI: 10.7326/0003-4819-117-4-303. View

Espinosa G, Bucciarelli S, Asherson R, Cervera R . Morbidity and mortality in the catastrophic antiphospholipid syndrome: pathophysiology, causes of death, and prognostic factors. Semin Thromb Hemost. 2008; 34(3):290-4. DOI: 10.1055/s-0028-1082274. View

Asherson R, Cervera R, Piette J, Font J, Lie J, Burcoglu A . Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998; 77(3):195-207. DOI: 10.1097/00005792-199805000-00005. View

Ruiz-Irastorza G, Hunt B, Khamashta M . A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies. Arthritis Rheum. 2007; 57(8):1487-95. DOI: 10.1002/art.23109. View

Schulman S, Svenungsson E, Granqvist S . Anticardiolipin antibodies predict early recurrence of thromboembolism and death among patients with venous thromboembolism following anticoagulant therapy. Duration of Anticoagulation Study Group. Am J Med. 1998; 104(4):332-8. DOI: 10.1016/s0002-9343(98)00060-6. View

Kitchens C . Thrombotic storm: when thrombosis begets thrombosis. Am J Med. 1998; 104(4):381-5. DOI: 10.1016/s0002-9343(98)00061-8. View

Ruiz-Irastorza G, Khamashta M, Hunt B, Escudero A, Cuadrado M, Hughes G . Bleeding and recurrent thrombosis in definite antiphospholipid syndrome: analysis of a series of 66 patients treated with oral anticoagulation to a target international normalized ratio of 3.5. Arch Intern Med. 2002; 162(10):1164-9. DOI: 10.1001/archinte.162.10.1164. View

10.

Asherson R, Cervera R, Piette J, Shoenfeld Y, Espinosa G, Petri M . Catastrophic antiphospholipid syndrome: clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore). 2001; 80(6):355-77. DOI: 10.1097/00005792-200111000-00002. View

11.

Cervera R, Khamashta M, Shoenfeld Y, Camps M, Jacobsen S, Kiss E . Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2008; 68(9):1428-32. DOI: 10.1136/ard.2008.093179. View

12.

Espinosa G, Cervera R, Asherson R . Catastrophic antiphospholipid syndrome and sepsis. A common link?. J Rheumatol. 2007; 34(5):923-6. View

13.

Erkan D, Yazici Y, Sobel R, Lockshin M . Primary antiphospholipid syndrome: functional outcome after 10 years. J Rheumatol. 2000; 27(12):2817-21. View

14.

Miyakis S, Lockshin M, Atsumi T, Branch D, Brey R, Cervera R . International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006; 4(2):295-306. DOI: 10.1111/j.1538-7836.2006.01753.x. View

15.

Asherson R, Cervera R, de Groot P, Erkan D, Boffa M, Piette J . Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003; 12(7):530-4. DOI: 10.1191/0961203303lu394oa. View

16.

Cervera R, Piette J, Font J, Khamashta M, Shoenfeld Y, Camps M . Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002; 46(4):1019-27. DOI: 10.1002/art.10187. View

17.

Ames P, Ciampa A, Margaglione M, Scenna G, Iannaccone L, Brancaccio V . Bleeding and re-thrombosis in primary antiphospholipid syndrome on oral anticoagulation: an 8-year longitudinal comparison with mitral valve replacement and inherited thrombophilia. Thromb Haemost. 2005; 93(4):694-9. DOI: 10.1160/TH04-11-0723. View

18.

Bucciarelli S, Espinosa G, Cervera R . The CAPS Registry: morbidity and mortality of the catastrophic antiphospholipid syndrome. Lupus. 2009; 18(10):905-12. DOI: 10.1177/0961203309106833. View

19.

Cervera R, Bucciarelli S, Espinosa G, Gomez-Puerta J, Ramos-Casals M, Shoenfeld Y . Catastrophic antiphospholipid syndrome: lessons from the "CAPS Registry"--a tribute to the late Josep Font. Ann N Y Acad Sci. 2007; 1108:448-56. DOI: 10.1196/annals.1422.047. View

20.

Bayraktar U, Erkan D, Bucciarelli S, Espinosa G, Asherson R . The clinical spectrum of catastrophic antiphospholipid syndrome in the absence and presence of lupus. J Rheumatol. 2007; 34(2):346-52. View