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Chapter 141 The Antiphospholipid Syndrome 2101
identified pathogenic mechanism of antiphospholipid-mediated defective
Main Points and Clinical Pearls—cont’d
placentation. Arthritis Rheum 62:1504–1512, 2010.
Erkan D, Aguiar CL, Andrade D, et al: 14th International Congress on
antibodies and antiphosphatidylserine antibodies may help to
clarify the picture. Antiphospholipid Antibodies: task force report on antiphospholipid
• Long-term warfarin anticoagulant therapy is the usual treatment syndrome treatment trends. Autoimmun Rev 13:685–696, 2014.
for thrombosis in patients with APS. The newer direct-acting oral Gropp K, Weber N, Reuter M, et al: beta(2)-glycoprotein I, the major target
anticoagulants (DOACs) have not been extensively evaluated in in antiphospholipid syndrome, is a special human complement regulator.
APS patients. Blood 118:2774–2783, 2011.
• Aspirin and heparin are recommended for pregnant APS patients Mekinian A, Lachassinne E, Nicaise-Roland P, et al: European registry of
and for prophylaxis against deep vein thrombosis during the babies born to mothers with antiphospholipid syndrome. Ann Rheum
postpartum period. Dis 72:217–222, 2013.
• CAPS patients have a high mortality and, in addition Mekinian A, Lazzaroni MG, Kuzenko A, et al: The efficacy of hydroxychloro-
to anticoagulants, often require plasmapheresis and
immunosuppressive agents. quine for obstetrical outcome in anti-phospholipid syndrome: Data from
• Asymptomatic patients with triple-positive aPL tests appear to a European multicenter retrospective study. Autoimmun Rev 14:498–502,
be at high risk for a first thrombotic event and for recurrence. 2015.
Consider treating prophylactically during high risk situations. Moulis G, Audemard-Verger A, Arnaud L, et al: Risk of thrombosis in patients
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SUGGESTED READINGS Proulle V, Furie RA, Merrill-Skoloff G, et al: Platelets are required for
enhanced activation of the endothelium and fibrinogen in a mouse
Agar C, de Groot PG, Morgelin M, et al: Beta2-glycoprotein I: a novel thrombosis model of APS. Blood 124:611–622, 2014.
component of innate immunity. Blood 2011. Pengo V, Ruffatti A, Legnani C, et al: Incidence of a first thromboem-
Agnelli G, Becattini C, Franco L: New oral anticoagulants for the treatment bolic event in asymptomatic carriers of high-risk antiphospholipid
of venous thromboembolism. Best Pract Res Clin Haematol 26:151–161, antibody profile: a multicenter prospective study. Blood 118:4714–4718,
2013. 2011.
Amengual O, Fujita D, Ota E, et al: Primary prophylaxis to prevent obstetric Pengo V, Banzato A, Bison E, et al: Antiphospholipid syndrome: critical
complications in asymptomatic women with antiphospholipid antibodies: analysis of the diagnostic path. Lupus 19:428–431, 2010.
a systematic review. Lupus 24(11):1135–1142, 2015. Perez-Sanchez C, Ruiz-Limon P, Aguirre MA, et al: Mitochondrial dysfunc-
Arnaud L, Mathian A, Devilliers H, et al: Patient-level analysis of five inter- tion in antiphospholipid syndrome: implications in the pathogenesis of the
national cohorts further confirms the efficacy of aspirin for the primary disease and effects of coenzyme Q(10) treatment. Blood 119:5859–5870,
prevention of thrombosis in patients with antiphospholipid antibodies. 2012.
Autoimmun Rev 14:192–200, 2015. Rand JH, Wu XX, Quinn AS, et al: Human monoclonal antiphospholipid
Andreoli L, Piantoni S, Dall’Ara F, et al: Vitamin D and antiphospholipid antibodies disrupt the annexin A5 anticoagulant crystal shield on phos-
syndrome. Lupus 21:736–740, 2012. pholipid bilayers: evidence from atomic force microscopy and functional
Brandt KJ, Fickentscher C, Boehlen F, et al: NF-kappaB is activated from assay. Am J Pathol 163:1193–1200, 2003.
endosomal compartments in antiphospholipid antibodies-treated human Ruiz-Irastorza G, Cuadrado MJ, Ruiz-Arruza I, et al: Evidence-based recom-
monocytes. J Thromb Haemost 12:779–791, 2014. mendations for the prevention and long-term management of thrombosis
Breen KA, Sanchez K, Kirkman N, et al: Endothelial and platelet mic- in antiphospholipid antibody-positive patients: report of a task force at
roparticles in patients with antiphospholipid antibodies. Thromb Res the 13th International Congress on antiphospholipid antibodies. Lupus
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Bezati E, Wu XX, Quinn AS, et al: A new trick for an ancient drug: quinine Schmidt-Tanguy A, Voswinkel J, Henrion D, et al: Antithrombotic effects
dissociates antiphospholipid immune complexes. Lupus 24:32–41, 2015. of hydroxychloroquine in primary antiphospholipid syndrome patients.
Canaud G, Bienaime F, Tabarin F, et al: Inhibition of the mTORC pathway J Thromb Haemost 11:1927–1929, 2013.
in the antiphospholipid syndrome. N Engl J Med 371:303–312, 2014. Sciascia S, Cosseddu D, Montaruli B, et al: Risk Scale for the diagnosis of
Cervera R, Rodriguez-Pinto I, Colafrancesco S, et al: 14th International Con- antiphospholipid syndrome. Ann Rheum Dis 70:1517–1518, 2011.
gress on Antiphospholipid Antibodies Task Force Report on Catastrophic Tanimura K, Jin H, Suenaga T, et al: beta2-glycoprotein I / HLA class II
Antiphospholipid Syndrome. Autoimmun Rev 13:699–707, 2014. complexes are novel autoantigens in antiphospholipid syndrome. Blood
Crowl A, Schullo-Feulner A, Moon JY: A Review of Warfarin Monitoring 125:2835–2844, 2015.
in Antiphospholipid Syndrome and Lupus Anticoagulant. Ann Pharma- Ueki H, Mizushina T, Laoharatchatathanin T, et al: Loss of maternal annexin
cother 2014. A5 increases the likelihood of placental platelet thrombosis and foetal loss.
de Jesus GR, Rodrigues G, de Jesus NR, et al: Pregnancy morbidity in Sci Rep 2:827, 2012.
antiphospholipid syndrome: what is the impact of treatment? Curr Wolgast LR, Arslan AA, Wu XX, et al: Reduction of annexin A5 anticoagulant
Rheumatol Rep 16:403, 2014. ratio identifies antiphospholipid antibody-positive patients with adverse
de Laat B, de Groot PG: Autoantibodies directed against domain I of beta2- clinical outcomes. J Thromb Haemost 2017, In Press.
glycoprotein I. Curr Rheumatol Rep 13:70–76, 2011. Xie H, Sheng L, Zhou H, et al: The role of TLR4 in pathophysiology
Di Simone N, Marana R, Castellani R, et al: Decreased expression of of antiphospholipid syndrome-associated thrombosis and pregnancy
heparin-binding epidermal growth factor-like growth factor as a newly morbidity. Br J Haematol 164:165–176, 2014.

