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A Young Disease: A Holistic Approach to the Treatment of Antiphospholipid Syndrome

Samantha C. Shapiro, MD  |  Issue: October 2022  |  September 8, 2022

Dr. Cervera: Fortunately, thrombocytopenia isn’t an issue when treating typical APS because platelets are rarely less than 70,000/mL. But in CAPS, platelet levels can be life-threateningly low. In those cases, first try to increase the platelets with glucocorticoids and IVIG. As soon as platelets are above 10,000–15,000, you can start low-dose heparin prophylaxis. When they reach 40,000 to 50,000, you can start full-dose anticoagulation.


Samantha C. Shapiro, MD, is an academic rheumatologist and an affiliate faculty member of the Dell Medical School at the University of Texas at Austin. She is also a member of the ACR Insurance Subcommittee.

References

  1. 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):1296–1304.
  2. 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):924–929.
  3. 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):281–291.
  4. Cuadrado MJ, Bertolaccini ML, Seed PT, et al. Low-dose aspirin vs. low-dose aspirin plus low-intensity warfarin in thromboprophylaxis: A prospective, multicentre, randomized, open, controlled trial in patients positive for antiphospholipid antibodies (ALIWAPAS). Rheumatology (Oxford). 2014 Feb;53(2):275–284.
  5. Ruiz-Irastorza G, Egurbide M v., Pijoan JI, et al. Effect of antimalarials on thrombosis and survival in patients with systemic lupus erythematosus. Lupus. 2006;15(9):577–583.
  6. 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):993–997.
  7. Ruiz-Irastorza G, Hunt BJ, Khamashta MA. A systematic review of secondary thromboprophylaxis in patients with antiphospholipid antibodies. Arthritis Rheum. 2007 Dec 15;57(8):1487–1495.
  8. Cervera R, Serrano R, Pons-Estel GJ, et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: A multicentre prospective study of 1000 patients. Ann Rheum Dis. 2015 Jun;74(6):1011-1018.
  9. Pengo V, Denas G, Zoppellaro G, et al. Rivaroxaban vs. warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018 Sep 17;132(13):1365–1371.
  10. Cervera R. CAPS registry. Lupus. 2012 Jun;21(7):755–757.
  11. Bucciarelli S, Espinosa G, Cervera R, et al. Mortality in the catastrophic antiphospholipid syndrome: Causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 2006 Aug;54(8):2568–2576.
  12. Asherson RA, Cervera R, Piette JC, et al. Catastrophic antiphospholipid syndrome: Clues to the pathogenesis from a series of 80 patients. Medicine (Baltimore). 2001 Nov;80(6):355–377.
  13. Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530­–534.
  14. Rodríguez-Pintó I, Espinosa G, Erkan D, et al. The effect of triple therapy on the mortality of catastrophic anti-phospholipid syndrome patients. Rheumatology (Oxford). 2018 Jul 1;57(7):1264–1270.
  15. Berman H, Rodríguez-Pintó I, Cervera R, et al. Rituximab use in the catastrophic antiphospholipid syndrome: Descriptive analysis of the CAPS registry patients receiving rituximab. Autoimmun Rev. 2013 Sep;12(11):1085–1090.
  16. López-Benjume B, Rodríguez-Pintó I, Amigo MC, et al. Eculizumab use in catastrophic antiphospholipid syndrome (CAPS): Descriptive analysis from the “CAPS Registry.” Autoimmun Rev. 2022 Apr;21(4):103055.

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Filed under:ConditionsMeeting ReportsOther Rheumatic Conditions Tagged with:Antiphospholipid Antibody Syndrome (APS)cardiovascularcatastrophic antiphospholipid syndromeEULARGlucocorticoidspatient care

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