Antiphospholipid syndrome

References

Key articles

Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006 Feb;4(2):295-306.Full text  Abstract

Barbhaiya M, Zuily S, Naden R, et al. 2023 ACR/EULAR antiphospholipid syndrome classification criteria. Ann Rheum Dis. 2023 Oct;82(10):1258-70.Full text  Abstract

Tektonidou MG, Andreoli L, Limper M, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis. 2019 Oct;78(10):1296-304.Full text  Abstract

Sammaritano LR, Bermas BL, Chakravarty EE, et al. 2020 American College of Rheumatology guideline for the management of reproductive health in rheumatic and musculoskeletal diseases. Arthritis Rheumatol. 2020 Apr;72(4):529-56.Full text  Abstract

American College of Obstetricians and Gynecologists. ACOG practice bulletin no. 132: antiphospholipid syndrome. Dec 2012 [internet publication].Full text

Reference articles

1. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006 Feb;4(2):295-306.Full text  Abstract

2. Barbhaiya M, Zuily S, Naden R, et al. 2023 ACR/EULAR antiphospholipid syndrome classification criteria. Ann Rheum Dis. 2023 Oct;82(10):1258-70.Full text  Abstract

3. Wilson WA, Gharavi AE, Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum. 1999 Jul;42(7):1309-11.Full text  Abstract

4. Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002 Apr;46(4):1019-27. Abstract

5. Duarte-García A, Pham MM, Crowson CS, et al. The epidemiology of antiphospholipid syndrome: a population-based study. Arthritis Rheumatol. 2019 Sep;71(9):1545-52. Abstract

6. Shi W, Krilis SA, Chong BH, et al. Prevalence of lupus anticoagulant and anticardiolipin antibodies in a healthy population. Aust N Z J Med. 1990 Jun;20(3):231-6. Abstract

7. Petri M. Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun. 2000 Sep;15(2):145-51. Abstract

8. Lim W, Crowther MA, Eikelboom JW. Management of antiphospholipid antibody syndrome: a systematic review. JAMA. 2006 Mar 1;295(9):1050-7.Full text  Abstract

9. Erkan D, Lockshin MD. What is antiphospholipid syndrome? Curr Rheumatol Rep. 2004 Dec;6(6):451-7. Abstract

10. Rai RS, Regan L, Clifford K, et al. Antiphospholipid antibodies and beta 2-glycoprotein-I in 500 women with recurrent miscarriage: results of a comprehensive screening approach. Hum Reprod. 1995 Aug;10(8):2001-5. Abstract

11. Loizou S, Byron MA, Englert HJ, et al. Association of quantitative anticardiolipin antibody levels with fetal loss and time of loss in systemic lupus erythematosus. Q J Med. 1988 Jul;68(255):525-31. Abstract

12. Jara LJ, Medina G, Vera-Lastra O, et al. The impact of gender on clinical manifestations of primary antiphospholipid syndrome. Lupus. 2005;14(8):607-12. Abstract

13. Meroni PL, Borghi MO, Raschi E, et al. Pathogenesis of antiphospholipid syndrome: understanding the antibodies. Nat Rev Rheumatol. 2011 Jun;7(6):330-9. Abstract

14. Mehdi AA, Uthman I, Khamashta M. Antiphospholipid syndrome: pathogenesis and a window of treatment opportunities in the future. Eur J Clin Invest. 2010 May;40(5):451-64. Abstract

15. Giannakopoulos B, Krilis SA. The pathogenesis of the antiphospholipid syndrome. N Engl J Med. 2013 Mar 14;368(11):1033-44. Abstract

16. de Laat B, Derksen RH, Urbanus RT, et al. IgG antibodies that recognize epitope Gly40-Arg43 in domain I of beta 2-glycoprotein I cause LAC, and their presence correlates strongly with thrombosis. Blood. 2005 Feb 15;105(4):1540-5. Abstract

17. Del Papa N, Raschi E, Catelli L, et al. Endothelial cells as a target for antiphospholipid antibodies: role of anti-beta 2 glycoprotein I antibodies. Am J Reprod Immunol. 1997 Sep;38(3):212-7. Abstract

18. Malia RG, Kitchen S, Greaves M, et al. Inhibition of activated protein C and its cofactor protein S by antiphospholipid antibodies. Br J Haematol. 1990 Sep;76(1):101-7. Abstract

19. Erkan D, Lockshin MD. New approaches for managing antiphospholipid syndrome. Nat Clin Pract Rheumatol. 2009 Mar;5(3):160-70. Abstract

20. Pierangeli SS, Vega-Ostertag ME, Gonzalez EB. New targeted therapies for treatment of thrombosis in antiphospholipid syndrome. Expert Rev Mol Med. 2007 Nov 13;9(30):1-15. Abstract

21. Salmon JE, Girardi G. Theodore E. Woodward Award: antiphospholipid syndrome revisited: a disorder initiated by inflammation. Trans Am Clin Climatol Assoc. 2007;118:99-114.Full text  Abstract

22. Girardi G, Berman J, Redecha P, et al. Complement C5a receptors and neutrophils mediate fetal injury in the antiphospholipid syndrome. J Clin Invest. 2003 Dec;112(11):1644-54.Full text  Abstract

23. Lynch AM, Murphy JR, Byers T, et al. Alternative complement pathway activation fragment Bb in early pregnancy as a predictor of preeclampsia. Am J Obstet Gynecol. 2008 Apr;198(4):385.e1-9.Full text  Abstract

24. Rand JH, Wu XX, Lapinski R, et al. Detection of antibody-mediated reduction of annexin A5 anticoagulant activity in plasmas of patients with the antiphospholipid syndrome. Blood. 2004 Nov 1;104(9):2783-90. Abstract

25. Rand JH, Wu XX. Antibody-mediated interference with annexins in the antiphospholipid syndrome. Thromb Res. 2004;114(5-6):383-9. Abstract

26. de Laat B, Wu XX, van Lummel M, et al. Correlation between antiphospholipid antibodies that recognize domain I of beta2-glycoprotein I and a reduction in the anticoagulant activity of annexin A5. Blood. 2007 Feb 15;109(4):1490-4. Abstract

27. Pierrot-Deseilligny Despujol C, Michel M, Khellaf M, et al. Antiphospholipid antibodies in adults with immune thrombocytopenic purpura. Br J Haematol. 2008 Aug;142(4):638-43.Full text  Abstract

28. Schreiber K, Sciascia S, de Groot PG, et al. Antiphospholipid syndrome. Nat Rev Dis Primers. 2018 Jan 11;4:17103. Abstract

29. Knight JS, Branch DW, Ortel TL. Antiphospholipid syndrome: advances in diagnosis, pathogenesis, and management. BMJ. 2023 Feb 27;380:e069717.Full text  Abstract

30. Ferreira E, Bettencourt PM, Moura LM. Valvular lesions in patients with systemic lupus erythematosus and antiphospholipid syndrome: an old disease but a persistent challenge. Rev Port Cardiol. 2012 Apr;31(4):295-9. Abstract

31. Tektonidou MG. Renal involvement in the antiphospholipid syndrome (APS)-APS nephropathy. Clin Rev Allergy Immunol. 2009;36:131-140. Abstract

32. Rodrigues CE, Carvalho JF, Shoenfeld Y. Neurological manifestations of antiphospholipid syndrome. Eur J Clin Invest. 2010 Apr;40(4):350-9. Abstract

33. Arnson Y, Shoenfeld Y, Alon E, et al. The antiphospholipid syndrome as a neurological disease. Semin Arthritis Rheum. 2010 Oct;40(2):97-108. Abstract

34. Ashrani AA, Heit JA. Incidence and cost burden of post-thrombotic syndrome. J Thromb Thrombolysis 2009 Nov;28(4):465-76. Abstract

35. Devreese KMJ, Ortel TL, Pengo V, et al. Laboratory criteria for antiphospholipid syndrome: communication from the SSC of the ISTH. J Thromb Haemost. 2018 Apr;16(4):809-13.Full text  Abstract

36. Brandt JT, Triplett DA, Alving B, et al. Criteria for the diagnosis of lupus anticoagulants: an update. On behalf of the Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the ISTH. Thromb Haemost. 1995 Oct;74(4):1185-90. Abstract

37. Pengo V, Tripodi A, Reber G, et al. Update of the guidelines for lupus anticoagulant detection. J Thromb Haemost. 2009 Oct;7(10):1737-40.Full text  Abstract

38. Tripodi A, Cohen H, Devreese KMJ. Lupus anticoagulant detection in anticoagulated patients. Guidance from the Scientific and Standardization Committee for lupus anticoagulant/antiphospholipid antibodies of the International Society on Thrombosis and Haemostasis. J Thromb Haemost. 2020 Jul;18(7):1569-75.Full text  Abstract

39. Devreese KM, Pierangeli SS, de Laat B, et al. Testing for antiphospholipid antibodies with solid phase assays: recommendations from the Subcommittee on Lupus Anticoagulant/Phospholipid-Dependent Antibodies. J Thromb Haemost. 2014 May;12(5):792-5. Abstract

40. Ruiz-Irastorza G, Crowther M, Branch W, et al. Antiphospholipid syndrome. Lancet. 2010 Oct 30;376(9751):1498-509. Abstract

41. Samanta D, Cobb S, Arya K. Sneddon syndrome: a comprehensive overview. J Stroke Cerebrovasc Dis. 2019 Aug;28(8):2098-108. Abstract

42. Abreu MM, Danowski A, Wahl DG, et al. The relevance of "non-criteria" clinical manifestations of antiphospholipid syndrome: 14th International Congress on Antiphospholipid Antibodies Technical Task Force Report on Antiphospholipid Syndrome Clinical Features. Autoimmun Rev. 2015 May;14(5):401-14. Abstract

43. Devreese KMJ, de Groot PG, de Laat B, et al. Guidance from the Scientific and Standardization Committee for lupus anticoagulant/antiphospholipid antibodies of the International Society on Thrombosis and Haemostasis: update of the guidelines for lupus anticoagulant detection and interpretation. J Thromb Haemost. 2020 Nov;18(11):2828-39.Full text  Abstract

44. Keeling D, Mackie I, Moore GW, et al. British Committee for Standards in Haematology. Guidelines on the investigation and management of antiphospholipid syndrome. Br J Haematol. 2012;157:47-58.Full text  Abstract

45. Tektonidou MG, Andreoli L, Limper M, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis. 2019 Oct;78(10):1296-304.Full text  Abstract

46. Derksen RH, de Groot PG, Kater L, et al. Patients with antiphospholipid antibodies and venous thrombosis should receive long term anticoagulant treatment. Ann Rheum Dis. 1993 Sep;52(9):689-92.Full text  Abstract

47. 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 Apr;104(4):332-8. Abstract

48. Crowther MA, Ginsberg JS, Julian J, et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003 Sep 18;349(12):1133-8.Full text  Abstract

49. Finazzi G, Marchioli R, Brancaccio V, et al. A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost. 2005 May;3(5):848-53.Full text  Abstract

50. Okuma H, Kitagawa Y, Yasuda T, et al. Comparison between single antiplatelet therapy and combination of antiplatelet and anticoagulation therapy for secondary prevention in ischemic stroke patients with antiphospholipid syndrome. Int J Med Sci. 2009 Dec 5;7(1):15-18.Full text  Abstract

51. Cohen H, Efthymiou M, Devreese KMJ. Monitoring of anticoagulation in thrombotic antiphospholipid syndrome. J Thromb Haemost. 2021 Apr;19(4):892-908.Full text  Abstract

52. Khairani CD, Bejjani A, Piazza G, et al. Direct oral anticoagulants vs vitamin K antagonists in patients with antiphospholipid syndromes: meta-analysis of randomized trials. J Am Coll Cardiol. 2023 Jan 3;81(1):16-30.Full text  Abstract

53. Medicines and Healthcare products Regulatory Agency. Direct-acting oral anticoagulants (DOACs): increased risk of recurrent thrombotic events in patients with antiphospholipid syndrome. June 2019 [internet publication].Full text

54. Pengo V, Denas G, Zoppellaro G, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018 Sep 27;132(13):1365-71.Full text  Abstract

55. Woller SC, Stevens SM, Kaplan D, et al. Apixaban compared with warfarin to prevent thrombosis in thrombotic antiphospholipid syndrome: a randomized trial. Blood Adv. 2022 Mar 22;6(6):1661-70.Full text  Abstract

56. Hamulyák EN, Scheres LJ, Marijnen MC, et al. Aspirin or heparin or both for improving pregnancy outcomes in women with persistent antiphospholipid antibodies and recurrent pregnancy loss. Cochrane Database Syst Rev. 2020 May 2;5:CD012852.Full text  Abstract

57. Sammaritano LR, Bermas BL, Chakravarty EE, et al. 2020 American College of Rheumatology guideline for the management of reproductive health in rheumatic and musculoskeletal diseases. Arthritis Rheumatol. 2020 Apr;72(4):529-56.Full text  Abstract

58. American College of Obstetricians and Gynecologists. ACOG practice bulletin no. 132: antiphospholipid syndrome. Dec 2012 [internet publication].Full text

59. Hunt BJ, Missfelder-Lobos H, Parra-Cordero M, et al. Pregnancy outcome and fibrinolytic, endothelial and coagulation markers in women undergoing uterine artery Doppler screening at 23 weeks. J Thromb Haemost. 2009 Jun;7(6):955-61.Full text  Abstract

60. Erkan D, Lockshin MD; APS ACTION members. APS ACTION - AntiPhospholipid Syndrome Alliance for Clinical Trials and International Networking. Lupus. 2012 Jun;21(7):695-8.Full text  Abstract

61. Hereng T, Lambert M, Hachulla E, et al. Influence of aspirin on the clinical outcomes of 103 anti-phospholipid antibodies-positive patients. Lupus. 2008 Jan;17(1):11-5. Abstract

62. Erkan D, Yazici Y, Peterson MG, et al. A cross-sectional study of clinical thrombotic risk factors and preventive treatments in antiphospholipid syndrome. Rheumatology (Oxford). 2002 Aug;41(8):924-9.Full text  Abstract

63. Erkan D, Harrison MJ, Levy R, et al. 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 Jul;56(7):2382-91.Full text  Abstract

64. Arnaud L, Mathian A, Ruffatti A, et al. Efficacy of aspirin for the primary prevention of thrombosis in patients with antiphospholipid antibodies: an international and collaborative meta-analysis. Autoimmun Rev. 2014 Mar;13(3):281-91. Abstract

65. Christiansen M, Grove EL, Hvas AM. Primary prevention of cardiovascular events with aspirin: toward more harm than benefit-a systematic review and meta-analysis. Semin Thromb Hemost. 2019 Jul;45(5):478-89. Abstract

66. Ziakas PD, Pavlou M, Voulgarelis M. Heparin treatment in antiphospholipid syndrome with recurrent pregnancy loss: a systematic review and meta-analysis. Obstet Gynecol. 2010 Jun;115(6):1256-62. Abstract

67. Aguiar CL, Erkan D. Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease. Ther Adv Musculoskelet Dis. 2013 Dec;5(6):305-14.Full text  Abstract

68. Kazzaz NM, McCune WJ, Knight JS. Treatment of catastrophic antiphospholipid syndrome. Curr Opin Rheumatol. 2016 May;28(3):218-27.Full text  Abstract

69. Erkan D. Expert perspective: management of microvascular and catastrophic antiphospholipid syndrome. Arthritis Rheumatol. 2021 Oct;73(10):1780-90. Abstract

70. Rubenstein E, Arkfeld DG, Metyas S, et al. Rituximab treatment for resistant antiphospholipid syndrome. J Rheumatol. 2006 Feb;33(2):355-7. Abstract

71. Tinti MG, Carnevale V, Inglese M, et al. Eculizumab in refractory catastrophic antiphospholipid syndrome: a case report and systematic review of the literature. Clin Exp Med. 2019 Aug;19(3):281-8. Abstract

72. Pleguezuelo DE, Díaz-Simón R, Cabrera-Marante O, et al. Case report: resetting the humoral immune response by targeting plasma cells with daratumumab in anti-phospholipid syndrome. Front Immunol. 2021;12:667515.Full text  Abstract

73. Erkan D, Vega J, Ramón G, et al. A pilot open-label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome. Arthritis Rheum. 2013 Feb;65(2):464-71.Full text  Abstract

74. ClinicalTrials.gov. Daratumumab in primary antiphospholipid syndrome (DARE-APS). ClinicalTrials.gov Identifier: NCT05671757. Jul 2024 [internet publication].Full text

75. Rajgopal R, Bear M, Butcher MK, et al. The effects of heparin and low molecular weight heparins on bone. Thromb Res. 2008;122(3):293-8. Abstract

76. Bouvier S, Cochery-Nouvellon E, Lavigne-Lissalde G, et al. Comparative incidence of pregnancy outcomes in treated obstetric antiphospholipid syndrome: the NOH-APS observational study. Blood. 2014 Jan 16;123(3):404-13. Abstract

77. Wallace DJ. Does hydroxychloroquine sulfate prevent clot formation in systemic lupus erythematosus? Arthritis Rheum. 1987 Dec;30(12):1435-6. Abstract

78. Schreiber K, Breen K, Cohen H, et al. Hydroxychloroquine to improve pregnancy outcome in women with antiphospholipid antibodies (HYPATIA) protocol: a multinational randomized controlled trial of hydroxychloroquine versus placebo in addition to standard treatment in pregnant women with antiphospholipid syndrome or antibodies. Semin Thromb Hemost. 2017 Sep;43(6):562-71.Full text  Abstract

79. Rand JH, Wu XX, Quinn AS, et al. Hydroxychloroquine directly reduces the binding of antiphospholipid antibody-beta2-glycoprotein I complexes to phospholipid bilayers. Blood. 2008 Sep 1;112(5):1687-95.Full text  Abstract

80. Kravvariti E, Koutsogianni A, Samoli E, et al. The effect of hydroxychloroquine on thrombosis prevention and antiphospholipid antibody levels in primary antiphospholipid syndrome: a pilot open label randomized prospective study. Autoimmun Rev. 2020 Apr;19(4):102491. Abstract

81. Kwon OC, Park YB, Park MC. Effect of statins on the prevention of recurrent thrombosis in thrombotic antiphospholipid syndrome. Rheumatology (Oxford). 2022 Apr 11;61(4):1548-55. Abstract

82. Glynn RJ, Danielson E, Fonseca FA, et al. A randomized trial of rosuvastatin in the prevention of venous thromboembolism. N Engl J Med. 2009 Apr 30;360(18):1851-61.Full text  Abstract

83. Kunutsor SK, Seidu S, Khunti K. Statins and primary prevention of venous thromboembolism: a systematic review and meta-analysis. Lancet Haematol. 2017 Feb;4(2):e83-93. Abstract

84. Yun Z, Duan L, Liu X, et al. An update on the biologics for the treatment of antiphospholipid syndrome. Front Immunol. 2023;14:1145145.Full text  Abstract

85. Sciascia S, Radin M, Cecchi I, et al. Open-label, prospective, phase II descriptive pilot trial of belimumab therapy for refractory and/or non-criteria manifestations of antiphospholipid syndrome: study protocol. Clin Exp Rheumatol. 2023 Mar;41(3):597-604.Full text  Abstract

86. ClinicalTrials.gov. IMPACT study: Improve pregnancy in APS with certolizumab therapy. ClinicalTrials.gov Identifier: NCT03152058. Oct 2023 [internet publication].Full text

87. Zuily S, Cohen H, Isenberg D, et al. Use of direct oral anticoagulants in patients with thrombotic antiphospholipid syndrome: Guidance from the Scientific and Standardization Committee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost. 2020 Sep;18(9):2126-37.Full text  Abstract

88. Arachchillage DRJ, Gomez K, Alikhan R, et al. Addendum to British Society for Haematology Guidelines on Investigation and Management of Antiphospholipid syndrome, 2012 (Br. J. Haematol. 2012; 157: 47-58): use of direct acting oral anticoagulants. Br J Haematol. 2020 Apr;189(2):212-5.Full text  Abstract

89. Khamashta MA, Cuadrado MJ, Mujic F, et al. The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med. 1995 Apr 13;332(15):993-7.Full text  Abstract

90. Kearon C, Gent M, Hirsh J, et al. A comparison of three months of anticoagulation with extended anticoagulation for a first episode of idiopathic venous thromboembolism. N Engl J Med. 1999 Mar 25;340(12):901-7. Abstract

91. Jepsen SY, Larsen JB, Christensen TD, et al. Warfarin monitoring and interference by lupus anticoagulant in patients with antiphospholipid syndrome. Thromb Res. 2022 Mar;211:127-32. Abstract

Use of this content is subject to our disclaimer