Skip to main content
SpringerLink
Log in
Book cover

Antiphospholipid Syndrome pp 247–266Cite as

Treatment of Non-criteria Manifestations in Antiphospholipid Syndrome

  • Chapter
  • First Online:

Abstract

Non-criteria manifestations of antiphospholipid syndrome (APS), such as skin ulcers, antiphospholipid antibody (aPL)-associated nephropathy, thrombocytopenia, and heart valve disease, represent a treatment challenge. Only few formal prospective studies address their pathogeneses and treatments. The management of some manifestations of APS remains empirical and with limited evidence-based data. In this chapter, together with a concise literature review, each situation is illustrated by a real clinical case followed by an expert discussion. Potential future treatment strategies for aPL-positive patients are also discussed.

This is a preview of subscription content, log in via an institution.

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   109.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   159.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Parsi K, Partsch H, Rabe E, Ramelet AA. Reticulate eruptions. Part 1: vascular networks and physiology. Aust J Dermatol. 2011;52:159–66.

    Article  Google Scholar 

  2. Parsi K, Partsch H, Rabe E, Ramelet AA. Reticulate eruptions. Part 2: historical perspectives, morphology, terminology and classification. Aust J Dermatol. 2011;52:237–44.

    Article  Google Scholar 

  3. Criado PR, Rivitti EA, Sotto MN, de Carvalho JF. Livedoid vasculopathy as a coagulation disorder. Autoimmun Rev. 2011;10:353–60.

    Article  PubMed  Google Scholar 

  4. Frances C. Dermatological manifestations of Hughes’ antiphospholipid antibody syndrome. Lupus. 2010;19:1071–7.

    Article  CAS  PubMed  Google Scholar 

  5. Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and pattern of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46:1019–27.

    Article  PubMed  Google Scholar 

  6. Erkan D, Lockshin MD. Non-criteria manifestations of antiphospholipid syndrome. Lupus. 2010;19:424–7.

    Article  CAS  PubMed  Google Scholar 

  7. Weinstein S, Piette W. Cutaneous manifestations of antiphospholipid antibody syndrome. Hematol Oncol Clin N Am. 2008;22:67–77.

    Article  Google Scholar 

  8. Cervera R, Tektonidou MG, Espinosa G, et al. Task force on catastrophic antiphospholipid syndrome (APS) and non-criteria APS manifestations (II): thrombocytopenia and skin manifestations. Lupus. 2011;20:174–81.

    Article  CAS  PubMed  Google Scholar 

  9. Drucker CR, Duncan WC. Antiplatelet therapy in atrophie blanche and livedo vasculitis. J Am Acad Dermatol. 1982;7:359–63.

    Article  CAS  PubMed  Google Scholar 

  10. Kern AB. Atrophie blanche: report of two patients treated with aspirin and dipyridamole. J Am Acad Dermatol. 1982;6:1048–53.

    Article  CAS  PubMed  Google Scholar 

  11. Asherson RA, Francès C, Iaccarino L, et al. The antiphospholipid antibody syndrome: diagnosis, skin manifestations and current therapy. Clin Exp Rheumatol. 2006;24:S46–51.

    CAS  PubMed  Google Scholar 

  12. Rossini J, Roverano S, Graf C, et al. Widespread cutaneous necrosis associated with antiphospholipid antibodies: report of four cases. J Clin Rheumatol. 2002;8:326–31.

    Article  PubMed  Google Scholar 

  13. Di Giacomo TB, Hussein TP, Souza DG, Criado PR. Frequency of thrombophilia determinant factors in patients with livedoid vasculopathy and treatment with anticoagulant drugs – a prospective study. J Eur Acad Dermatol Venereol. 2010;24:1340–6.

    Article  PubMed  Google Scholar 

  14. Weishaupt C, Strölin A, Kahle B, et al. Anticoagulation with rivaroxaban for livedoid vasculopathy (RILIVA): a multicentre, single-arm, open-label, phase 2a, proof-of-concept trial. Lancet Haematol. 2016;3:e72–9.

    Article  PubMed  Google Scholar 

  15. Frances C, Niang S, Laffitte E, et al. Dermatologic manifestations of the antiphospholipid syndrome: two hundred consecutive cases. Arthritis Rheum. 2005;52:1785–93.

    Article  PubMed  Google Scholar 

  16. Gertner E. Treatment with sildenafil for the healing of refractory skin ulcerations in the antiphospholipid syndrome. Lupus. 2003;12:133–5.

    Article  CAS  PubMed  Google Scholar 

  17. Srinivasan SK, Pittelkow MR, Cooper Jr LT. Recombinant tissue plasminogen activator for the treatment of cutaneous infarctions in antiphospholipid antibody syndrome: a case report. Angiology. 2001;52:635–9.

    Article  CAS  PubMed  Google Scholar 

  18. Bounfour T, Bouaziz JD, Bézier M, et al. Intravenous immunoglobulins is difficult-to-treat ulcerated livedoid vasculopathy: five cases and review in literature. Int J Dermatol. 2013;52:1135–9.

    Google Scholar 

  19. Monshi B, Posch C, Vujic I, Sesti A, Sobotka S, Rappersberger K. Efficacy of intravenous immunoglobulins in livedoid vasculopathy: long-term follow-up of 11 patients. J Am Acad Dermatol. 2014;71:738–44.

    Google Scholar 

  20. Tenedios F, Erkan D, Lockshin MD. Rituximab in the primary antiphospholipid syndrome (PAPS). Arthritis Rheum. 2005;52:4078.

    Google Scholar 

  21. Costa R, Fazal S, Kaplan RB, et al. Successful plasma exchange combined with rituximab therapy in aggressive APS-related cutaneous necrosis. Clin Rheumatol. 2013;32:S79–82.

    Article  PubMed  Google Scholar 

  22. 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;65:464–71.

    Google Scholar 

  23. Vasconcelos R, Criado PR, Belda Jr W. Livedoid vasculopathy secondary to high levels of lipoprotein(a). Br J Dermatol. 2011;164:1111–3.

    Google Scholar 

  24. Goerge T, Weishaupt C, Metze D, et al. Livedoid vasculopathy in a pediatric patient with elevated lipoprotein(a) levels: prompt response to continuous low-molecular-weight heparin. Arch Dermatol. 2010;146:927–8.

    Google Scholar 

  25. Gantcheva M. Dermatologic aspects of antiphospholipid syndrome. Int J Dermatol. 1998;37:173–80.

    Article  CAS  PubMed  Google Scholar 

  26. Diógenes MJ, Diógenes PC, de Morais Carneiro RM, et al. Cutaneous manifestations associated with antiphospholipid antibodies. Int J Dermatol. 2004;43:632–7.

    Google Scholar 

  27. Callen JP. Livedoid vasculopathy: what it is and how the patient should be evaluated and treated. Arch Dermatol. 2006;142:1481–2.

    Google Scholar 

  28. Alavi A, Hafner J, Dutz JP, et al. Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach. J Am Acad Dermatol. 2013;69:1033–42.

    Google Scholar 

  29. Ray R, Sharma A, Vasudevan B, Sridhar J, et al. Livedoid vasculopathy with hyperhomocysteinemia responding to hyperbaric Oxygen therapy. Indian J Dermatol. 2015;60:524.

    Google Scholar 

  30. Criado PR, de Souza Espinell DP, Valentef NS, et al. Livedoid vasculopathy and high levels of lipoprotein (a): response to danazol. Dermatol Ther. 2015;28:248–53.

    Google Scholar 

  31. Kim EJ, Yoon SY, Park HS, et al. Pulsed intravenous immunoglobulin therapy in refractory ulcerated livedoid vasculopathy: seven cases and a literature review. Dermatol Ther. 2015;28:287–90.

    Google Scholar 

  32. Polo Gascón MR, de Carvalho JF, de Souza Espinel DP, et al. Quality-of-life impairment in patients with livedoid vasculopathy. J Am Acad Dermatol. 2014;71:1024–6.

    Google Scholar 

  33. Cuadrado MJ, Mujic F, Muñoz E, et al. Thrombocytopenia in the antiphospholipid syndrome. Ann Rheum Dis. 1997;56:194–6.

    Google Scholar 

  34. Finazzi G. The Italian registry of antiphospholipid antibodies. Haematologica. 1997;82:101–5.

    Google Scholar 

  35. Forastiero R. Bleeding in the antiphospholipid syndrome. Hematology. 2012;17(Suppl 1):S153–5.

    CAS  PubMed  Google Scholar 

  36. Artim-Esen B, Diz-Küçükkaya R, İnanc M. The significance and management of thrombocytopenia in antiphospholipid syndrome. Curr Rheumatol Rep. 2015;17:14.

    Article  PubMed  Google Scholar 

  37. Alarcón-Segovia D, Cabral AR. Antiphospholipid antibodies. Where do they come from? Where do they go? J Rheumatol. 1994;21:982–9.

    Google Scholar 

  38. Macchi L, Rispal P, Clofent-Sanchez G, et al. Anti-platelet antibodies in patients with systemic lupus erythematosus and the primary antiphospholipid antibody syndrome: their relationship with the observed thrombocytopenia. Br J Haematol. 1997;98:336–41.

    Google Scholar 

  39. Scully M, Hunt BJ, Benjamin S,et al, British Committee for Standards in Haematology. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol. 2012;158:323–35.

    Google Scholar 

  40. Alarcón-Segovia D, Sánchez-Guerrero J. Correction of thrombocytopenia with small dose aspirin in the primary antiphospholipid syndrome. J Rheumatol. 1989;16:1359–61.

    Google Scholar 

  41. Durand JM, Lefevre P, Kaplanski G, et al. Correction of thrombocytopenia with dapsone in the primary antiphospholipid syndrome. J Rheumatol. 1993;20:1777–8.

    Google Scholar 

  42. Suarez IM, Diaz RA, Aguayo Canela D, et al. Correction of severe thrombocytopenia with chloroquine in the primary antiphospholipid syndrome. Lupus. 1996;5:81–3.

    Google Scholar 

  43. Pons I, Espinosa G, Cervera R. Efficacy and safety of rituximab in the treatment of primary antiphospholipid syndrome: analysis of 24 cases from the bibliography review. Med Clin (Barc). 2015;144:97–104.

    Google Scholar 

  44. Delgado Alves J, Inanc M, Diz-Kucukkaya R, et al. Thrombotic risk in patients submitted to splenectomy for systemic lupus erythematosus and antiphospholipid antibody syndrome-related thrombocytopenia. Eur J Intern Med. 2004;15:162–7.

    Google Scholar 

  45. Atsumi T, Furukawa S, Amengual O, et al. Antiphospholipid antibody associated thrombocytopenia and the paradoxical risk of thrombosis. Lupus. 2005;14:499–504.

    Google Scholar 

  46. Wig S, Chan M, Thachil J, et al. A case of relapsing and refractory catastrophic anti-phospholipid syndrome successfully managed with eculizumab, a complement 5 inhibitor. Rheumatology (Oxford). 2016;55:382–4.

    Google Scholar 

  47. Bakhtar O, Thajudeen B, Braunhut BL, et al. A case of thrombotic microangiopathy associated with antiphospholipid antibody syndrome successfully treated with eculizumab. Transplantation. 2014;98(3):17–8.

    Google Scholar 

  48. Sperati CJ, Streiff MB. Acute renal failure in a patient with antiphospholipid syndrome and immune thrombocytopenic purpura treated with eltrombopag. Am J Hematol. 2010;85:724–6.

    Google Scholar 

  49. Boulon C, Vircoulon M, Constans J. Eltrombopag in systemic lupus erythematosus with antiphospholipid syndrome: thrombotic events. Lupus. 2016;25:331.

    Google Scholar 

  50. LaMoreaux B, Barbar-Smiley F, Ardoin S, Madhoun H. Two cases of thrombosis in patients with antiphospholipid antibodies during treatment of immune thrombocytopenia with romiplostim, a thrombopoietin receptor agonist. Semin Arthritis Rheum. 2016;45:e10–2.

    Google Scholar 

  51. Pazzola G, Zuily S, Erkan D.The challenge of bleeding in antiphospholipid antibody-positive patients.Curr Rheumatol Rep. 2015;17:7.

    Google Scholar 

  52. Diz-Küçükkaya R, Lopez JA. Thrombocytopenia, chapter 117. In: Kaushansky K, Lichtman MA, Prchol JT, Levi MM, Press OW, Burns LJ, Caligiuri MA, editors. Williams hematology. 9th ed. New York: Mc Graw Hill Company; 2016.

    Google Scholar 

  53. Serpatwari A, Bennett D, Logie JW, et al. Thromboembolic events among adult patients with primary immune thrombocytopenia in the United Kingdom general practice research database. Haematologica. 2010;95:1167–75.

    Article  Google Scholar 

  54. Diz-Kucukkaya R, Hacihanefioglu A, Yenerel M, et al. Antiphospholipid antibodies and antiphospholipid syndrome in patients presenting with immune thrombocytopenic purpura: a prospective cohort study. Blood. 2001;98:1760–4.

    Google Scholar 

  55. Stasi R, Stipa E, Masi M, et al. Prevalence and clinical significance of elevated antiphospholipid antibodies in patients with idiopatic thrombocytopenic purpura. Blood. 1994;84:4203–8.

    CAS  PubMed  Google Scholar 

  56. Asherson RA, Cervera R, Merrill JT, Erkan D. Antiphospholipid antibodies and the antiphospholipid syndrome: clinical significance and treatment. Semin Thromb Hemost. 2008;34:256–66.

    Article  CAS  PubMed  Google Scholar 

  57. Marie I, Maurey G, Herve F, et al. Intravenous immunoglobulin-associated arterial and venous thrombosis, report of a series and review of the literature. Br J Dermatol. 2006;155:714–21.

    Article  CAS  PubMed  Google Scholar 

  58. Jansen AJ, Swart RM, te Boekhorst PA. Thrombopoietin receptor agonists for immune thrombocytopenia. N Engl J Med. 2011;365:2240–1.

    Article  PubMed  Google Scholar 

  59. Mehta TP, Smythe MA, Mattson JC. Strategies for managing heparin therapy in patients with antiphosholipid antibody syndrome. Pharmacotherapy. 2011;31:1221–31.

    Article  CAS  PubMed  Google Scholar 

  60. Tenedios F, Erkan D, Lockshin MD. Cardiac involvement in the antiphospholid syndrome. Lupus. 2005;14:691–6.

    Article  CAS  PubMed  Google Scholar 

  61. Long BR, Leya F. The role of antiphospholipid syndrome in cardiovascular disease. Hematol Oncol Clin North Am. 2008;22:79–94.

    Article  PubMed  Google Scholar 

  62. Cervera R. Recent advances in antiphospholipid antibody-related valvulopathies. J Autoimmun. 2005;15:123–5.

    Article  Google Scholar 

  63. Espinola-Zavaleta N, Vargas-Barron J, Colmenares-Galvis T, et al. Echocardiographic evaluation of patients with primary antiphospholipid syndrome. Am Heart J. 1999;137:974–9.

    Article  Google Scholar 

  64. Zuily S, Regnault V, Selton-Suty C, et al. Increased risk for heart valve disease associated with antiphospholipid antibodies in patients with systemic lupus erythematosus: meta-analysis of echocardiographic P. Circulation. 2011;124:215–24.

    Article  CAS  PubMed  Google Scholar 

  65. Cervera R, Tektonidou MG, Espinosa G, et al. Task force on catastrophic antiphospholipid syndrome (APS) and non-criteria APS manifestations (I): catastrophic APS, APS nephropathy and heart valve lesions. Lupus. 2011;20:165–73.

    Article  CAS  PubMed  Google Scholar 

  66. Appropriatness criteria for TTE and TEE. Am Coll Cardiol J Am Soc Echocardiogr. 2007;20:787–805.

    Google Scholar 

  67. Espinola-Zavaleta N, Amigo MC, Vargas-Barron J, et al. Two- and three-dimensional echocardiography in primary antiphospholipid syndrome: misdiagnosis as rheumatic valve disease. Lupus. 2001;10:511–3.

    Article  CAS  PubMed  Google Scholar 

  68. Shroff H, Benenstein R, Freedberg R, Mehl S, Saric M. Mitral valve Libman–Sacks endocarditis visualized by real time three-dimensional transesophageal echocardiography. Echocardiography. 2012;29:E100.

    Article  PubMed  Google Scholar 

  69. Roldan CA, Qualls CR, Sopko KS, Sibbitt WL Jr. Transthoracic versus transesophageal echocardiography for detection of Libman-Sacks endocarditis: a randomized controlled study. J Rheumatol 2008;35:224–229.

    Google Scholar 

  70. Mavrogeni SI, Sfikakis PP, Kitas GD, Kolovou G, Tektonidou MG. Cardiac involvement in antiphospholipid syndrome: the diagnostic role of noninvasive cardiac imaging. Semin Arthritis Rheum. 2016;45:611–6.

    Article  CAS  PubMed  Google Scholar 

  71. Espinola-Zavaleta N, Montes RM, Soto ME, et al. Primary antiphospholipidsyndrome: a 5 year transesophageal echocardiographic follow up study. J Rheumatol. 2004;31:2402–7.

    Google Scholar 

  72. Morita H, Daido H, Suwa T, et al. Overt congestive heart failure with mitral and aortic regurgitation due to antiphospholipid syndrome in a patient with systemic lupus erythematosus. Intern Med. 2000;39:506–11.

    Google Scholar 

  73. Berkun Y, Elami A, Meir K, et al. Increased morbidity and mortality in patients with antiphospholipid syndrome undergoing valve replacement surgery. J Thorac Cardiovasc Surg. 2004;127(2):414–20.

    Google Scholar 

  74. Erdozain J-G, Ruiz-Irastorza G, Segura M-I, et al. Cardiac valve replacement in patients with antiphospholipid syndrome. Arthritis Care Res. 2012;64:1256–60.

    Google Scholar 

  75. Bert JS, Abdullah M, Dahle TG, et al. Transcatheter aortic valve replacement for advanced valvular disease in active SLE and APS. Lupus. 2013;22:1046.

    Article  CAS  PubMed  Google Scholar 

  76. Krause I, Lev S, Fraser A, et al. Close association between valvar heart disease and central nervous system manifestations in the antiphospholipid syndrome. Ann Rheum Dis. 2005;64:1490–3.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  77. Roldan CA, Gelgand EA, Qualls CR, Sibbitt Jr WL. Valvular heart disease as a cause of cerebrovascular disease in patients with systemic lupus erythematosus. Am J Cardiol. 2005;95:1441–7.

    Article  PubMed  Google Scholar 

  78. Pardos-Gea J, Ordi-Ros J, Avegliano G, et al. Echocardiography at diagnosis of antiphospholipid syndrome provides prognostic information on valvular disease evolution and identifies two subtypes of patients. Lupus. 2010;19:575–82.

    Article  CAS  PubMed  Google Scholar 

  79. Urbanus RT, Siegerink B, Roest M, et al. Antiphospholipid antibodies and risk of myocardial infarction and ischaemic stroke in young women in the RATIO study: a case-control study. Lancet Neurol. 2009;8:998–1005.

    Article  CAS  PubMed  Google Scholar 

  80. Roach RE, Helmerhorst FM, Lijfering WM, Stijnen T, Algra A, Dekkers OM. Combined oral contraceptives: the risk of myocardial infarction and ischemic stroke Cochrane Database Syst Rev. 2015; Art CD 011054.

    Google Scholar 

  81. 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;4:295–306.

    Article  CAS  PubMed  Google Scholar 

  82. Pengo V, Ruffatti A, Legnani C, et al. Incidence of a first thromboembolic event in asymptomatic carriers of high-risk antiphospholipid antibody profile: a multicenter prospective study. Blood. 2011;118:4714–8.

    Article  CAS  PubMed  Google Scholar 

  83. Pengo V, Ruiz-Irastorza G, Denas G, et al. High intensity anticoagulation in the prevention of the recurrence of arterial thrombosis in antiphospholipid syndrome: ‘PROS’ and ‘CONS’. Autoimmun Rev. 2012;11:577–80.

    Article  PubMed  Google Scholar 

  84. Ruiz-Irastorza G, Cuadrado MJ, Ruiz-Arruza I, et al. Evidence-based recommendations for the prevention and long-term management of thrombosis in antiphospholipid antibody-positive patients: report of a task force at the 13th International Congress on antiphospholipid antibodies. Lupus. 2011;20:206–18.

    Article  CAS  PubMed  Google Scholar 

  85. Erkan D, Aguiar CL, Andrade D, et al. 14th International Congress on Antiphospholipid Antibodies task force report on antiphospholipid syndrome treatment trends. Autoimmun Rev. 2014;13:685–96.

    Article  CAS  PubMed  Google Scholar 

  86. Legault KJ, Ugarte A, Crowther MA, Irastorza G. Prevention of recurrent thrombosis in antiphospholipid syndrome: different from the general population? Curr Rheumatol Rep. 2016;18:26.

    Article  PubMed  Google Scholar 

  87. Wang J, Ning R, Wang Y. Plasma D-dimer level, the promising prognostic biomarker forthe acute cerebral infarction patients. J Stroke Cerebrovasc Dis. 2016;25:2011–5.

    Article  PubMed  Google Scholar 

  88. Sadanaga T, Sadanaga M, Ogawa S. Evidence that D-dimer levels predict subsequent thromboembolic and cardiovascular events in patients with atrial fibrillation during oral anticoagulant therapy. J Am Coll Cardiol. 2010;55:2225–31.

    Article  CAS  PubMed  Google Scholar 

  89. Sciascia S, Giachino O, Roccatello D. Prevention of thrombosis relapse in antiphospholipid syndrome patients refractory to conventional therapy using intravenous immunoglobulin. Clin Exp Rheumatol. 2012;30:409–13.

    CAS  PubMed  Google Scholar 

  90. Nesher G, Ilany J, Rosenmann D, Abraham AS. Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features and treatment. Semin Arthritis Rheum. 1997;27:27–35.

    Article  CAS  PubMed  Google Scholar 

  91. Petri MA. Classification criteria for antiphospholipid syndrome: the case for cardiac valvular disease. J Rheumatol. 2004;31:2329–30.

    PubMed  Google Scholar 

  92. Turiel M, Sarzi-Puttini P, Peretti R, et al. Five-year follow-up by transesophageal echocardiographic studies in primary antiphospholipid syndrome. Am J Cardiol. 2005;96:574–9.

    Article  PubMed  Google Scholar 

  93. Kampolis C, Tektonidou M, Moyssakis I, Tzelepis GE, Moutsopoulos H. Evolution of cardiac dysfunction in patients with antiphospholipid antibodies and/or antiphospholipid syndrome: a 10-year follow-up study. Semin Arthritis Rheum. 2014;43:558–65.

    Article  CAS  PubMed  Google Scholar 

  94. Kernan W, Ovbiagele B, Black H, on behalf of the American Heart Association Stroke Council, Council on Cardiovascular and Stroke Nursing, Council on Clinical Cardiology, and Council on Peripheral Vascular Disease. Guidelines for the prevention of stroke in patients with stroke and transient ischemic attack: a guideline for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2014;45:2160–236.

    Article  PubMed  Google Scholar 

  95. Nochy D, Daugas E, Droz D, et al. The intrarenal vascular lesions associated with primary antiphospholipid syndrome. J Am Soc Nephrol. 1999;10:507–18.

    CAS  PubMed  Google Scholar 

  96. Tektonidou MG, Sotsiou F, Nakopoulou L, Vlachoyiannopoulos PG, Moutsopoulos HM. Antiphospholipid syndrome nephropathy in patients with systemic lupus erythematosus and antiphospholipid antibodies: prevalence, clinical associations, and long-term outcome. Arthritis Rheum. 2004;50:2569–79.

    Article  PubMed  Google Scholar 

  97. Tektonidou MG, Sotsiou F, Moutsopoulos HM. Antiphospholipid syndrome (APS) nephropathy in catastrophic, primary, and systemic lupus erythematosus-related APS. J Rheumatol. 2008;35:1983–8.

    Google Scholar 

  98. Dayal NA, Isenberg DA. Endstage renal failure in primary antiphospholipid syndrome-case report and review of literature. Rheumatology (Oxford). 2003;42:1128–9.

    Article  CAS  Google Scholar 

  99. Korkmaz C, Kabukcuoglu S, Isiksoy S, et al. Renal involvement in primary antiphospholipid syndrome and its response to immunosuppressive therapy. Lupus. 2003;12:760–5.

    Article  CAS  PubMed  Google Scholar 

  100. Erkan D, Lockshin MD. New approaches for managing antiphospholipid syndrome. Nat Clin Pract Rheumatol. 2009;5:160–70.

    Article  PubMed  Google Scholar 

  101. Kronbichler A, Mayer G. Renal involvement in autoimmune connective tissue diseases. BMC. 2013;11:95.

    Article  CAS  Google Scholar 

  102. Sciascia S, Cuadrado MJ, Khamashta M, et al. Renal involvement in antiphospholipid syndrome. Nat Rev Nephrol. 2014;10:279–89.

    Article  CAS  PubMed  Google Scholar 

  103. Velik-Salchner C, Lederer W, Wiedermann F. Eculizumab and renal transplantation in a patient with catastrophic antiphospholipid syndrome: effect of heparin on complement activation. Lupus. 2011;20:772.

    Article  CAS  PubMed  Google Scholar 

  104. Wu LH, Yu F, Tan Y, et al. Inclusion of renal vascular lesions in the 2003 ISN/RPS system for classifying lupus nephritis improves renal outcome predictions. Kidney Int. 2013;83:715–23.

    Article  CAS  PubMed  Google Scholar 

  105. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association-European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71:1771–82.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  106. 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;14:401–14.

    Article  PubMed  Google Scholar 

  107. Kleinknecht D, Bobrie G, Meyer O, Noël LH, Callard P, Ramdane M. Recurrent thrombosis and renal vascular disease in patients with a lupus anticoagulant. Nephrol Dial Transplant. 1989;4:854–8.

    Article  CAS  PubMed  Google Scholar 

  108. D'Agati V, Kunis C, Williams G, Appel GB. Anti-cardiolipin antibody and renal disease: a report three cases. J Am Soc Nephrol. 1990;1:777–84.

    PubMed  Google Scholar 

  109. Tsagalis G, Psimenou E, Nakopoulou L, Laggouranis A. Effective treatment of antiphospholipid syndrome with plasmapheresis and rituximab. Hippokratia. 2010;14:215–6.

    CAS  PubMed  PubMed Central  Google Scholar 

  110. Seshan SV, Franzke CW, Redecha P, Monestier M, Mackman N, Girardi G. Role of tissue factor in a mouse model of thrombotic microangiopathy induced by antiphospholipid antibodies. Blood. 2009;114:1675–83.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  111. Andrade D, Tektonidou M. Emerging therapies in antiphospholipid syndrome. Curr Rheumatol Rep. 2016;18:22.

    Article  PubMed  Google Scholar 

  112. Lonze BE, Zachary AA, Magro CM, et al. Eculizumab prevents recurrent antiphospholipid antibody syndrome and enables successful renal transplantation. Am J Transplant. 2014;14:459–65.

    Article  CAS  PubMed  Google Scholar 

  113. Canaud G, Kamar N, Anglicheau D, et al. Eculizumab improves posttransplant thrombotic microangiopathy due to antiphospholipid syndrome recurrence but fails to prevent chronic vascular changes. Am J Transplant. 2013;13:2179–85.

    Article  CAS  PubMed  Google Scholar 

  114. Canaud G, Bienaimé F, Tabarin F, et al. Inhibition of the mTORC pathway in the antiphospholipid syndrome. N Engl J Med. 2014;371:303–12.

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Rheumatology Division, São Paulo University, Brazil, São Paulo, Brazil

    Michelle Remião Ugolini-Lopes

  2. Department of Dermatology, Hospital das clinicas da Faculdade de Medicina da Universidade de Sao Paulo, Santo Andre, Sao Paulo, Brazil

    Paulo Ricardo Criado

  3. Department of Dermatology, St. Vincent’s Hospital, Sydney, Darlinghurst, NSW, Australia

    Kurosh Parsi

  4. Department of Dermatology, Phlebology and Fluid Mechanics Research Laboratory, St. Vincent’s Centre for Applied Medical Research, University of New South Wales (UNSW), Sydney, Australia

    Kurosh Parsi

  5. Hematology and Oncology, Medical Corner, Nisantasi, Istanbul, Turkey

    Reyhan Diz Kucukkaya

  6. Department of Rheumatology, ABC Medical Center, Mexico City, Mexico

    Mary-Carmen Amigo

  7. Rheumatology Unit, Laiko Hospital, First Department of Propaedeutic Internal Medicine, Athens, Greece

    Maria G. Tektonidou

  8. Department of Rheumatology, Hospital Das Clinicas, Sao Paulo, Brazil

    Danieli Andrade

Authors
  1. Michelle Remião Ugolini-Lopes
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Paulo Ricardo Criado
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Kurosh Parsi
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Reyhan Diz Kucukkaya
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mary-Carmen Amigo
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Maria G. Tektonidou
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Danieli Andrade
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Michelle Remião Ugolini-Lopes .

Editor information

Editors and Affiliations

  1. The Barbara Volcker Center for Women and Rheumatic Diseases, Associate Attending Physician, Hospital for Special Surgery, Associate Professor of Medicine, Weill Cornell Medicine, New York, New York, USA

    Doruk Erkan

  2. The Barbara Volcker Center for Women and Rheumatic Diseases, Attending Physician, Hospital for Special Surgery, Professor of Medicine and Obstetrics-Gynecology, Weill Cornell Medicine, New York, New York, USA

    Michael D. Lockshin

Rights and permissions

Reprints and permissions

Copyright information

© 2017 Springer International Publishing AG

About this chapter

Cite this chapter

Ugolini-Lopes, M.R. et al. (2017). Treatment of Non-criteria Manifestations in Antiphospholipid Syndrome. In: Erkan, D., Lockshin, M. (eds) Antiphospholipid Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-319-55442-6_13

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-55442-6_13

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-55440-2

  • Online ISBN: 978-3-319-55442-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation