Difference between revisions of "Antiphospholipid antibody syndrome"
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==== Anticoagulation ==== | ==== Anticoagulation ==== | ||
− | * [[Warfarin (Coumadin)]] PO with INR goal 2 | + | * [[Warfarin (Coumadin)]] PO with INR goal 2 to 3.0 |
* [[Aspirin]] 100 mg PO once per day | * [[Aspirin]] 100 mg PO once per day | ||
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''Note: TRAPS was closed prematurely due to excess events in the rivaroxaban arm.'' | ''Note: TRAPS was closed prematurely due to excess events in the rivaroxaban arm.'' | ||
====Anticoagulation==== | ====Anticoagulation==== | ||
− | *[[Warfarin (Coumadin)]] PO titrated to goal INR 2 | + | *[[Warfarin (Coumadin)]] PO titrated to goal INR 2 to 3.0 |
**Note: some trials specify a goal INR 2.5 without a range given | **Note: some trials specify a goal INR 2.5 without a range given | ||
Revision as of 12:19, 13 May 2021
Section editor | |
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Benjamin Tillman, MD Vanderbilt University Nashville, TN |
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Guidelines
Emerging data suggests the DOACs are inferior to VKAs in patients with APS. The trials are included here for reference, but most do not recommend these agents for APS. Due to the paucity of trials in this particular condition, many seek guidance from the venous thromboembolism (VTE) literature.
"How I Treat"
- 2021: Cohen & Isenberg How I treat anticoagulant-refractory thrombotic antiphospholipid syndrome
All lines of therapy
Aspirin & Warfarin
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Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Okuma et al. 2009 | Phase III (E-esc) | Aspirin | Lower cumulative incidence of ischemic stroke |
Note: Small study of 20 patients. All meeting the 2006 Sydney criteria for APS in setting of an ischemic stroke.
Anticoagulation
- Warfarin (Coumadin) PO with INR goal 2 to 3.0
- Aspirin 100 mg PO once per day
Continued indefinitely
References
- Okuma H, Kitagawa Y, Yasuda T, Tokuoka K, Takagi S. 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):15-8. link to original article link to PMC article PubMed
Rituximab monotherapy
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Regimen
Study | Evidence | Efficacy |
---|---|---|
Erkan et al. 2013 (RITAPS) | Phase II | Some improvement in non-criteria manifestations of APS |
Note: This small phase 2 study (19 patients) did not identify any significant change in the antiphospholipid antibody profiles of the enrolled patients, but it did observe some improvements in the non-criteria manifestations of APS. This included thrombocytopenia, cardiac valve disease, skin ulcer, aPL nephropathy, and cognitive dysfunction.
Immunosuppressive therapy
- Rituximab (Rituxan) 1000 mg IV once on day 1
14-day cycle for 2 cycles
References
- RITAPS: Erkan D, Vega J, Ramon G, Kozora E, Lockshin MD. A pilot open-label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome. Arthritis Rheum. 2013 Feb;65(2):464-71. link to original article PubMed
Rivaroxaban monotherapy
Regimen
Study | Evidence | Comparator | Efficacy |
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Cohen et al. 2016 (RAPS) | Phase II/III (E-switch-ooc) | Warfarin | Inconclusive whether non-inferior % change in endogenous thrombin potential |
Pengo et al. 2018 (TRAPS) | Phase III (E-switch-ooc) | Warfarin | Higher rate of events |
Note: TRAPS was closed prematurely due to excess events in the rivaroxaban arm. Although we include here for historical context, this regimen should not be used outside of the context of a clinical trial.
Anticoagulation
- Rivaroxaban (Xarelto) PO 20 mg PO once per day
Continued indefinitely
References
- RAPS: Cohen H, Hunt BJ, Efthymiou M, Arachchillage DR, Mackie IJ, Clawson S, Sylvestre Y, Machin SJ, Bertolaccini ML, Ruiz-Castellano M, Muirhead N, Doré CJ, Khamashta M, Isenberg DA; RAPS trial investigators. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol. 2016 Sep;3(9):e426-36. link to original article link to PMC article PubMed
- TRAPS: Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffatti A, Andreoli L, Tincani A, Cenci C, Prisco D, Fierro T, Gresele P, Cafolla A, De Micheli V, Ghirarduzzi A, Tosetto A, Falanga A, Martinelli I, Testa S, Barcellona D, Gerosa M, Banzato A. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018 Sep 27;132(13):1365-1371. Epub 2018 Jul 12. link to original article PubMed
Warfarin monotherapy
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Regimen
Study | Evidence | Comparator | Efficacy |
---|---|---|---|
Crowther et al. 2003 | Phase III (C) | High-intensity Warfarin (INR 3.1 to 4.0) | Did not meet primary endpoint of recurrent VTE |
Finazzi et al. 2005 (WAPS) | Phase III (C) | High-intensity Warfarin (INR 3 to 4.5) | Did not meet primary endpoint of recurrent symptomatic VTE |
Cohen et al. 2016 (RAPS) | Phase II/III (C) | Rivaroxaban | Inconclusive whether non-inferior % change in endogenous thrombin potential |
Pengo et al. 2018 (TRAPS) | Phase III (C) | Rivaroxaban | Fewer events |
Ordi-Ros et al. 2019 (SAP-02) | Phase II/III (C) | Rivaroxaban | Inconclusive whether non-inferior new VTE |
Note: TRAPS was closed prematurely due to excess events in the rivaroxaban arm.
Anticoagulation
- Warfarin (Coumadin) PO titrated to goal INR 2 to 3.0
- Note: some trials specify a goal INR 2.5 without a range given
Continued indefinitely
References
- Crowther MA, Ginsberg JS, Julian J, Denburg J, Hirsh J, Douketis J, Laskin C, Fortin P, Anderson D, Kearon C, Clarke A, Geerts W, Forgie M, Green D, Costantini L, Yacura W, Wilson S, Gent M, Kovacs MJ. 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. Erratum in: N Engl J Med. 2004 Jul 8;351(2):200. N Engl J Med. 2003 Dec 25;349(26):2577. link to original article PubMed
- WAPS: Finazzi G, Marchioli R, Brancaccio V, Schinco P, Wisloff F, Musial J, Baudo F, Berrettini M, Testa S, D'Angelo A, Tognoni G, Barbui T. 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. link to original article PubMed
- RAPS: Cohen H, Hunt BJ, Efthymiou M, Arachchillage DR, Mackie IJ, Clawson S, Sylvestre Y, Machin SJ, Bertolaccini ML, Ruiz-Castellano M, Muirhead N, Doré CJ, Khamashta M, Isenberg DA; RAPS trial investigators. Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol. 2016 Sep;3(9):e426-36. link to original article link to PMC article PubMed
- TRAPS: Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffatti A, Andreoli L, Tincani A, Cenci C, Prisco D, Fierro T, Gresele P, Cafolla A, De Micheli V, Ghirarduzzi A, Tosetto A, Falanga A, Martinelli I, Testa S, Barcellona D, Gerosa M, Banzato A. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018 Sep 27;132(13):1365-1371. Epub 2018 Jul 12. link to original article PubMed
- SAP-02: Ordi-Ros J, Sáez-Comet L, Pérez-Conesa M, Vidal X, Riera-Mestre A, Castro-Salomó A, Cuquet-Pedragosa J, Ortiz-Santamaria V, Mauri-Plana M, Solé C, Cortés-Hernández J. Rivaroxaban versus vitamin K antagonist in antiphospholipid syndrome: a randomized noninferiority trial. Ann Intern Med. 2019 Oct 15. [Epub ahead of print] link to original article PubMed