Skip to main content
SpringerLink
Log in

Antiphospholipid Syndrome

  • Chapter
  • First Online:
Precision Anticoagulation Medicine

  • 1007 Accesses

Abstract

Antiphospholipid syndrome (APS) comprises a myriad of venous, arterial obstetric, or valvular heart involvement with high serum levels of antiphospholipid antibodies (aPL). Catastrophic antiphospholipid syndrome (CAPS) is associated with high morbidity and mortality. Correlation with other immune disorders especially systemic lupus erythematosus (SLE) can occur. Therapy includes anticoagulation and anti-inflammatory/immunomodulatory medications.

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

Access this chapter

Log in via an institution
eBook
USD 16.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight 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

Institutional subscriptions

Similar content being viewed by others

Abbreviations

aCL:

Anticardiolipin antibodies

ADAMTS-13:

A disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13

ADP:

Adenosine diphosphate

anti-PF4:

Anti-platelet factor 4

aPL:

Antiphospholipid antibodies

ApoER2:

Apolipoprotein E receptor 2

APS:

Antiphospholipid syndrome

ASA:

Aspirin

CAPS:

Catastrophic antiphospholipid syndrome

CD:

Cluster of differentiation

CNS:

Central nervous system

Cox:

Cyclooxygenase

DIC:

Disseminated intravascular clotting

ELISA:

Enzyme-linked immunosorbent assay;

F:

Coagulation factor

GAPSS:

Global antiphospholipid syndrome score

GPL:

IgG aPL units

HELLP:

Hemolysis, elevated liver enzymes, and low platelet syndrome

HIT:

Heparin-induced thrombocytopenia

HLA:

Human leucocyte antigen

HMG-Co A:

β-hydroxy β-methylglutaryl-CoA

Ig:

Immunoglobulin

INR:

International normalized ratio

IVIG:

Intravenous immunoglobulins

LMWH:

Low molecular weight heparin

LRP8:

Low-density lipoprotein receptor-related protein 8

MPL:

IgM aPL units

mTOR:

Mammalian target of rapamycin

PTT:

Partial thromboplastin time

SLE:

Systemic lupus erythematosus

TLR:

Toll-like receptor

TTP-HUS:

Thrombotic thrombocytopenic purpura–hemolytic uremic syndrome

Xa:

Activated factor X

References

  1. Corban MT, Duarte-Garcia A, McBane RD, Matteson EL, Lerman LO, Lerman A. Antiphospholipid syndrome. Role of vascular endothelial cells and implications for risk stratification and targeted therapeutics. J Am Coll Cardiol. 2017;69(18):2317–30.

    Article  PubMed  Google Scholar 

  2. Chighizola CB, Andreoli L, Gerosa M, Tincani A, Ruffatti A, Meroni PL. The treatment of anti-phospholipid syndrome: a comprehensive clinical approach. J Autoimmun. 2018a;90:1–27. https://doi.org/10.1016/j.jaut.2018.02.003.

    Article  CAS  PubMed  Google Scholar 

  3. Gómez-Puerta JA, Cervera R. Diagnosis and classification of the antiphospholipid syndrome. J Autoimmun. 2014;48–49:20–5.

    Article  PubMed  CAS  Google Scholar 

  4. Lockshin MD. Update on antiphospholipid syndrome. Bull NYU Hosp Jt Dis. 2008;66(3):195–7.

    PubMed  Google Scholar 

  5. Durcan L, Petri M. Epidemiology of the antiphospholipid syndrome. In Handbook of systemic autoimmune diseases. Vol. 12. Elsevier Ltd. 2016; p. 17–30. (Handbook of systemic autoimmune diseases) https://doi.org/10.1016/B978-0-444-63655-3.00002-8.

    Google Scholar 

  6. Meroni PL, Borghi MO, Raschi E, Tedesco F. Pathogenesis of antiphospholipid syndrome: understanding the antibodies. Nat Rev Rheumatol. 2011;7(6):330–9.

    Article  CAS  PubMed  Google Scholar 

  7. Sacharidou A, Chambliss KL, Ulrich V, Salmon JE, Shen YM, Herz J, et al. Antiphospholipid antibodies induce thrombosis by PP2A activation via apoER2-Dab2-SHC1 complex formation in endothelium. Blood. 2018;131(19):2097–110.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Chighizola CB, Ubiali T, Meroni PL. Treatment of thrombotic antiphospholipid syndrome: the rationale of current management—an insight into future approaches. J Immunol Res. 2015a;2015:Article ID 951424.

    Article  Google Scholar 

  9. Groot PG, Meijers JC. β(2)-glycoprotein I: evolution, structure and function. J Thromb Haemost. 2011;9(7):1275–84.

    Article  PubMed  CAS  Google Scholar 

  10. Meroni PL, Chighizola CB, Rovelli F, Gerosa M. Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers. Arthritis Res Ther. 2014;16:209.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Movva S (Chief ed. Diamond, HS). Antiphosphopholipid syndrome Medscape. https://emedicine.medscape.com/article/333221-clinical. 2017.

  12. Miyakis S, Lockshin MD, Atsumi T, , Branch DW, Brey RL, Cervera R et al 2006. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006, (4):295–306.

    Article  Google Scholar 

  13. Hughes GR, Khamashta MA. Seronegative antiphospholipid syndrome. Ann Rheum Dis. 2003;62(12):1127.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Rodriguez-Garcia JL, Bertolaccini ML, Cuadrado MJ, Sanna G, Ateka-Barrutia O, Khamashta MA. Clinical manifestations of antiphospholipid syndrome (APS) with and without antiphospholipid antibodies (the so-called ‘seronegative APS’). Ann Rheum Dis. 2012;71(2):242–4.

    Article  PubMed  Google Scholar 

  15. Sciascia S, Amigo MC, Roccatello D, Khamashta M. Diagnosing antiphospholipid syndrome: ‘extra-criteria’ manifestations and technical advances. Nat Rev Rheumatol. 2017;13(9):548.

    Article  CAS  PubMed  Google Scholar 

  16. Zohoury N, Bertolaccini ML, Rodriguez-Garcia JL, Shums Z, Ateka-Barrutia O, Sorice M, Norman GL, Khamashta M. Closing the serological gap in the antiphospholipid syndrome: the value of “non-criteria” antiphospholipid antibodies. J Rheumatol. 2017;44(11):1597–602.

    Article  CAS  PubMed  Google Scholar 

  17. Uthman I, Noureldine MH, Ruiz-Irastorza G, Khamashta M. Management of antiphospholipid syndrome. Ann Rheum Dis. 2019;78(2):155–61.

    Article  CAS  PubMed  Google Scholar 

  18. Hughes GR, Khamashta MA. ‘Seronegative antiphospholipid syndrome’: an update. Lupus. 2019;28(3):273–4. https://doi.org/10.1177/0961203319826358.

    Article  CAS  PubMed  Google Scholar 

  19. Cohen D, Berger SP, Steup-Beekman GM, Bloemenkamp KWM, Bajema IM. Diagnosis and management of the antiphospholipid syndrome. BMJ. 2010;340:c2541.

    Article  PubMed  Google Scholar 

  20. Mendoza-Pinto C, García-Carrasco M, Cervera R. Role of infectious diseases in the antiphospholipid syndrome (including its catastrophic variant). Curr Rheumatol Rep. 2018;20(10):62.

    Article  PubMed  CAS  Google Scholar 

  21. Dlott JS, Roubey RA. Drug-induced lupus anticoagulants and antiphospholipid antibodies. Curr Rheumatol Rep. 2012;14(1):71–8.

    Article  CAS  PubMed  Google Scholar 

  22. Vassalo J, Spector N, de Meis E, Rabello LS, Rosolem MM, do Brasil PE, et al. Antiphospholipid antibodies in critically ill patients with cancer: a prospective cohort study. J Crit Care. 2014;29:533–8.

    Article  PubMed  Google Scholar 

  23. Gómez-Puerta JA, Espinosa G, Cervera R. Antiphospholipid antibodies. From general concepts to its relation with malignancies. Antibodies. 2016;5:18. https://doi.org/10.3390/antib5030018.

    Article  CAS  PubMed Central  Google Scholar 

  24. Sebastiani Domenico G, Minisola G, Galeazzi M. HLA class II alleles and genetic predisposition to the antiphospholipid syndrome. Autoimmun Rev. 2003;2(6):387–94.

    Article  CAS  Google Scholar 

  25. Musio F, Bohen EM, Yuan CM, Welch PG. Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum. 1998;28:1–19.

    Article  CAS  PubMed  Google Scholar 

  26. Ortel TL, Erkan D, Kitchens CS. How I treat catastrophic thrombotic syndromes. Blood. 2015;126:1285–93.

    Article  CAS  PubMed  Google Scholar 

  27. Abu-Zeinah G, Oromendia C, De Sancho MT. Thrombotic risk factors, antithrombotic therapy, and outcomes of asymptomatic carriers of antiphospholipid antibodies and patients with antiphospholipid syndrome. Blood. 2017;130:3710.

    Google Scholar 

  28. Chighizola CB, Andreoli L, de Jesus GR, Banzato A, Benanzato A, Pons-Estel GJ, Erkan D, APS ACTION. The association between antiphospholipid antibodies and pregnancy morbidity, stroke, myocardial infarction, and deep vein thrombosis: a critical review of the literature. Lupus. 2015b;24(9):980–4.

    Article  CAS  PubMed  Google Scholar 

  29. Pengo V, Ruffatti A, Legnani C, Testa S, Fierro T, Marongiu F, 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 

  30. Ruffatti A, Tonello M, Visentin MS, Bontadi A, Hoxha A, De Carolis S, et al. Risk factors for pregnancy failure in patients with anti-phospholipid syndrome treated with conventional therapies: a multicentre case–control study. Rheumatology. 2011;50:1684–9.

    Article  CAS  PubMed  Google Scholar 

  31. Forastiero R. Multiple antiphospholipid antibodies positivity and antiphospholipid syndrome criteria re-evaluation. Lupus. 2014;23(12):1252–4.

    Article  CAS  PubMed  Google Scholar 

  32. Giron-Gonzalez JA, Garcıa del Rıo E, Rodrıguez C, Rodrıguez-Martorell J, Serrano A. Antiphospholipid syndrome and asymptomatic carriers of antiphospholipid antibody: prospective ~analysis of 404 individuals. J Rheumatol. 2004;31(8):1560–7.

    PubMed  Google Scholar 

  33. Mustonen P, Lehtonen KV, Javela K, Puurunen M. Persistent anti- phospholipid antibody (aPL) in asymptomatic carriers as a risk factor for future thrombotic events: a nationwide prospective study. Lupus. 2014;23(14):1468–76.

    Article  CAS  PubMed  Google Scholar 

  34. Pengo V, Testa S, Martinelli I, Ghirarduzzi A, Legnani C, Gresele P, et al. Incidence of a first thromboembolic event in carriers of isolated lupus anticoagulant. Thromb Res. 2015;135(1):46–9.

    Article  CAS  PubMed  Google Scholar 

  35. Galli M, Borrelli G, Jacobsen EM, Marfisi RM, Finazzi G, Marchioli R, et al. Clinical significance of different antiphospholipid antibodies in the WAPS (warfarin in the anti- phospholipid syndrome) study. Blood. 2007;110(4):1178–83.

    Article  CAS  PubMed  Google Scholar 

  36. Kearon C, Parpia S, Spencer FA, Baglin T, Stevens SM, Bauer KA, et al. Antiphospholipid antibodies and recurrent thrombosis after a first unprovoked venous thromboembolism. Blood. 2018;131(19):2151–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  37. Sciascia S, Sanna G, Murru V, Roccatello D, Khamashta MA, Bertolaccini ML. GAPSS: the global anti-phospholipid syndrome score. Rheumatology (Oxford). 2013a;52:1397403.

    Article  Google Scholar 

  38. Sciascia S, Bertolaccini ML, Roccatello D, Khamashta MA. Independent validation of the antiphospholipid score for the diagnosis of antiphospholipid syndrome. Ann Rheum Dis. 2013b;72:142–3.

    Article  PubMed  Google Scholar 

  39. Sciascia S, Baldovino S, Schreiber K, Solfietti L, Radin M, Cuadrado MJ, Menegatti E, Erkan D, Roccatello D. Thrombotic risk assessment in antiphospholipid syndrome: the role of new antibody specificities and thrombin generation assay. Clin Mol Allergy. 2016a;14:6.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  40. Oku K, Amengual O, Bohgaki T, Horita T, Yasuda S, Atsumi T. An independent validation of the global anti-phospholipid syndrome score in a Japanese cohort of patients with autoimmune diseases. Lupus. 2015;24:774–5.

    Article  CAS  PubMed  Google Scholar 

  41. Zuily S, de Laat B, Mohamed S, Kelchtermans H, Shums Z, Albesa R, et al. Validity of the global anti-phospholipid syndrome score to predict thrombosis: a prospective multicentre cohort study. Rheumatology. 2015;54(11):2071–5.

    Article  CAS  PubMed  Google Scholar 

  42. Islam A, Alam F, Wong KK, Kamal MA, Gan SH. Thrombotic management of antiphospholipid syndrome: towards novel targeted therapies. Curr Vasc Pharmacol. 2017;15(4):313–26.

    Article  CAS  PubMed  Google Scholar 

  43. Tektonidou MG, Andreoli L, Limper M, Amoura Z, Cervera R, Costedoat-Chalumeau N, et al. EULAR recommendations for the management of antiphospholipid syndrome in adults. Ann Rheum Dis. 2019:1–9. https://doi.org/10.1136/annrheumdis-2019-215213.

    Article  PubMed  Google Scholar 

  44. Skeith L. Anticoagulating patients with high-risk acquired thombophilias. Blood. 2018;132:439–49.

    Article  Google Scholar 

  45. Kearon C, Iorio A, Palareti G, Subcommittee on Control of Anti-coagulation of the SSC of the ISTH. Risk of recurrent venous thromboembolism after stopping treatment in cohort studies: recommendation for acceptable rates and standardized reporting. J Thromb Haemost. 2010;8(10):2313–5.

    Article  CAS  PubMed  Google Scholar 

  46. Carrier M, Le Gal G, Wells PS, Rodger MA. Systematic review: case-fatality rates of recurrent venous thromboembolism and major bleeding events among patients treated for venous thromboembolism. Ann Intern Med. 2010;152(9):578–89.

    Article  PubMed  Google Scholar 

  47. Kearon C, Akl EA, Comerota AJ, Prandoni P, Bounameaux H, Goldhaber SZ, et al. Antithrombotic therapy for VTE disease: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence- Based Clinical Practice Guidelines. Chest. 2012;141:e419S–94S.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  48. Khamashta MA, Cuadrado MJ, Mujic F, Taub NA, Hunt BJ, Hughes GR. The management of thrombosis in the antiphospholipid-antibody syndrome. N Engl J Med. 1995;332(15):993–7.

    Article  CAS  PubMed  Google Scholar 

  49. Pengo V, Denas G, Zoppellaro G, Jose SP, Hoxha A, Ruffatti A, et al. Rivaroxaban vs warfarin in high-risk patients with antiphospholipid syndrome. Blood. 2018;132(13):1365–71.

    Article  CAS  PubMed  Google Scholar 

  50. Goldhaber SZ, Eriksson H, Kakkar A, Schellong S, Feuring M, Fraessdorf M, et al. Efficacy of dabigatran versus warfarin in patients with acute venous thromboembolism in the presence of thrombophilia: findings from RE-COVER®, RE- COVERII, and RE-MEDY. Vasc Med. 2016;21(6):506–14.

    Article  CAS  PubMed  Google Scholar 

  51. Alijotas-Reig J, Miguel-Moncín MS, Cisteró-Bahíma A. Aspirin desensitization in the treatment of antiphospholipid syndrome during pregnancy in ASA-sensitive patients. Am J Reprod Immunol. 2006 Jan;55(1):45–50.

    Article  CAS  PubMed  Google Scholar 

  52. Comarmond C, Jego P, Veyssier-Belot C, Marie I, Mekinian A, Elmaleh-Sachs A, et al. Cessation of oral anticoagulants in antiphospholipid syndrome. Lupus. 2017;26(12):1291–6. https://doi.org/10.1177/0961203317699285.

    Article  CAS  PubMed  Google Scholar 

  53. Garcia D, Erkan D. Diagnosis and management of anti-phospholipid syndrome. NEJM. 2018;378(21):2010–21.

    Article  CAS  PubMed  Google Scholar 

  54. Chighizola CB, Shoenfeld Y, Meroni PL. Therapy for antiphospholipid miscarriages: throwing the baby out with the bathwater? Am J Reprod Immunol. 2018b;79(3):e12792.

    Article  CAS  Google Scholar 

  55. Schreiber K, Sciascia S, de Groot PG, Devreese K, Jacobsen S, Ruiz-Irastorza G, et al. Antiphospholipid syndrome. Nat Rev Dis Primers. 2018;4:Article number: 17103.

    Article  Google Scholar 

  56. Laskin CA, Spitzer KA, Clark CA, Crowther MR, Ginsberg JS, Hawker GA, et al. Low molecular weight heparin and aspirin for recurrent pregnancy loss: results from the randomized, controlled HepASA trial. J Rheumatol. 2009;36(2):279–87. https://doi.org/10.3899/jrheum.080763.

    Article  CAS  PubMed  Google Scholar 

  57. Rey E, Garneau P, David M, Gauthier R, Leduc L, Michon N, et al. Dalteparin for the prevention of recurrence of placental-mediated complications of pregnancy in women without thrombophilia: a pilot randomized controlled trial. J Thromb Haemost. 2009;7:58–64.

    Article  CAS  PubMed  Google Scholar 

  58. Fischer-Betz R, Specker C, Brinks R, Schneider M. Pregnancy outcome in patients with antiphospholipid syndrome after cerebral ischaemic events: an observational study. Lupus. 2012;21:1183–9.

    Article  CAS  PubMed  Google Scholar 

  59. Sciascia S, Hunt BJ, Talavera-Garcia E, Lliso G, Khamashta MA, Cuadrado MJ. The impact of hydroxychloroquine treatment on pregnancy outcome in women with antiphospholipid antibodies. Am J Obstet Gynecol. 2016b;214(2):273.e1–8.

    Article  CAS  Google Scholar 

  60. Lefkou E, Mamopoulos A, Dagklis T, Vosnakis C, Rousso D, Girardi G. Pravastatin improves pregnancy outcomes in obstetric antiphospholipid syndrome refractory to antithrombotic therapy. J Clin Invest. 2016;126(8):2933–40. https://doi.org/10.1172/JCI86957.

    Article  PubMed  PubMed Central  Google Scholar 

  61. Cervera R, Serrano R, Pons-Estel GJ, Ceberio-Hualde L, Shoenfeld Y, de Ramón E, Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies), 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;74:1011–8.

    Article  CAS  PubMed  Google Scholar 

  62. Zuily S, Regnault V, Selton-Suty C, Eschwège V, Bruntz JF, Bode-Dotto E, et al. Increased risk for heart valve disease associated with antiphospholipid antibodies in patients with systemic lupus erythematosus: meta-analysis of echocardiographic studies. Circulation. 2011;124:215–24.

    Article  CAS  PubMed  Google Scholar 

  63. Ziporen L, Goldberg I, Arad M, Hojnik M, Ordi-Ros J, Afek A, et al. Libman-Sacks endocarditis in the antiphospholipid syndrome: immunopathologic findings in deformed heart valves. Lupus. 1996;5:196–205.

    Article  CAS  PubMed  Google Scholar 

  64. Zuily S, Huttin O, Mohamed S, Marie PY, Selton-Suty C, Wahl D. Valvular heart disease in antiphospholipid syndrome. Curr Rheumatol Rep. 2013;15:320.

    Article  PubMed  CAS  Google Scholar 

  65. Roldan CA, Sibbitt WL, Qualls CR, Jung RE, Greene ER, Gasparovic CM, et al. Libman-Sacks endocarditis and embolic cerebrovascular disease. J Am Coll Cardiol Img. 2013;6:973.

    Article  Google Scholar 

  66. Asherson R, Cervera R, de Groot P, Erkan D, Boffa MC, Piette JC, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530–4.

    Article  CAS  PubMed  Google Scholar 

  67. Cusick MF, Libbey JE, Fujinami RS. Molecular mimicry as a mechanism of autoimmune disease. Clin Rev Allergy Immunol. 2012;42(1):102–11.

    Article  CAS  PubMed  Google Scholar 

  68. Barratt-Due A, Fløisand Y, Orrem HL, Kvam AK, Holme PA, Bergseth G, et al. Complement activation is a crucial pathogenic factor in catastrophic antiphospholipid syndrome. Rheumatology (Oxford). 2016;55(7):1337–9.

    Article  Google Scholar 

  69. George JN, Chen Q, Deford CC, Al-Nouri Z. Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency. J Clin Apher. 2012;27:302–11.

    Article  PubMed  Google Scholar 

  70. Cervera R, Rodríguez-Pintó I, Espinosa G. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: a comprehensive review. J Autoimmun. 2018;92:1–11.

    Article  PubMed  Google Scholar 

  71. Cervera R, Rodríguez-Pintó I, Colafrancesco S, Conti F, Valesini G, Rosário C, et al. 14th international congress on antiphospholipid antibodies task force report on catastrophic antiphospholipid syndrome. Autoimmun Rev. 2014;13(7):699–707.

    Article  PubMed  Google Scholar 

  72. Gansner JM, Berliner N. The rheumatology/hematology interface: CAPS and MAS diagnosis and management. Hematology. 2018;2018:313–7.

    Article  PubMed  PubMed Central  Google Scholar 

  73. Crowley MP, Cuadrado MJ, Hunt BJ. Catastrophic antiphospholipid syndrome on switching from warfarin to rivaroxaban. Thromb Res. 2017;153:37–9.

    Article  CAS  PubMed  Google Scholar 

  74. Rodríguez-Pintó I, Espinosa G, Erkan D, Shoenfeld Y, Cervera R, CAPS Registry Project Group. The effect of triple therapy on the mortality of catastrophic anti-phospholipid syndrome patients. Rheumatology. 2018;57:1264–70.

    Article  PubMed  Google Scholar 

  75. Ruffatti A, De Silvestro G, Marson P, Tonello M, Calligaro A, Favaro M, et al. Catastrophic antiphospholipid syndrome: lessons from 14 cases successfully treated in a single center. A narrative report. J Autoimmun. 2018;93:124–30.

    Article  CAS  PubMed  Google Scholar 

  76. Zanatta E, Cozzi M, Marson P, Cozzi F. The role of plasma exchange in the management of autoimmune disorders. Br J Hematol. 2019; https://doi.org/10.1111/bjh.15903.

  77. Marson P, Monti G, Montani F, Riva A, Mascia MT, Castelnovo L, et al. Apheresis treatment of cryoglobulinemic vasculitis: a multicentre cohort study of 159 patients. Transfus Apher Sci. 2018;57:639–45.

    Article  CAS  PubMed  Google Scholar 

  78. Rodrıguez-Pinto I, Moitinho M, Santacreu I, Shoenfeld Y, Erkan D, Espinosa G, CAPS Registry Project Group (European Forum on Antiphospholipid Antibodies), et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the International CAPS Registry. Autoimmun Rev. 2016;15(12):1120–4.

    Article  PubMed  CAS  Google Scholar 

  79. Sukara G, Baresic M, Sentic M, Brcic L, Anic B. Catastrophic anti- phospholipid syndrome associated with systemic lupus erythematosus treated with rituximab: case report and a review of the literature. Acta Reumatol Port. 2015;40(2):169–75.

    CAS  PubMed  Google Scholar 

  80. Dogru A, Ugan Y, Şahin M, Karahan N, Tunç ŞE. Catastrophic anti- phospholipid syndrome treated with rituximab: a case report. Eur J Rheumatol. 2017;4(2):145–7.

    Article  PubMed  PubMed Central  Google Scholar 

  81. Rymarz A, Niemczyk S. The complex treatment including rituximab in the Management of Catastrophic Antiphospholid Syndrome with renal involvement. BMC Nephrol. 2018;19:132.

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  82. Yan SF, Mackman N, Kisiel W, Stern DM, Pinsky DJ. Hypoxia/hypoxemia-induced activation of the procoagulant pathways and the pathogenesis of ischemia-associated thrombosis. Arterioscler Thromb Vasc Biol. 1999;19(2):2029–35.

    Article  CAS  PubMed  Google Scholar 

  83. Schobersberger W, Fries D, Mittermayr M, Innerhofer P, Sumann G, Schobersberger B, et al. Changes of biochemical markers and functional tests for clot formation during long-haul flights. Thromb Res. 2002;108(1):19–24.

    Article  PubMed  Google Scholar 

  84. Aldington S, Pritchard A, Perrin K, James K, Wijesinghe M, Beasley R. Prolonged seated immobility at work is a common risk factor for venous thromboembolism leading to hospital admission. Int Med J. 2008;38(2):133–5.

    Article  CAS  Google Scholar 

  85. Silverman D, Gendreau M. Medical issues associated with commercial flights. Lancet. 2009;373(9680):2067–77.

    Article  PubMed  PubMed Central  Google Scholar 

  86. Geerts WH, Bergqvist D, Pineo GF, James K, Wijesinghe M, Beasley R, et al. Prevention of venous thromboembolism: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines (8th edition). Chest. 2008;133(6 Suppl):381S–453S.

    Article  CAS  PubMed  Google Scholar 

  87. Cesarone MR, Becaro G, Nicolaides AN, Incandela L, De S, Geroulakos G, et al. Venous thrombosis from air travel: the LONGFLIT3 study—prevention with aspirin vs low-molecular-weight heparin (LMWH) in high-risk subjects: a randomized trial. Angiology. 2002;53(1):1–6.

    Article  PubMed  Google Scholar 

  88. Sandhu VK, Teves K. Antiphospholipid syndrome: the risk of travel at high altitudes. Rheumatologist. 2018;. www.the-rheumatologist.org/article/antiphospholipid-syndrome-the-risk-of-travel-at-high-altitudes.

Download references

Author information

Authors and Affiliations

  1. Internal Medicine and Hematology, Cairo University, Giza, Egypt

    Mervat Mattar

  2. Division of Clinical Immunology and Rheumatology, University of Alabama at Birmingham, Birmingham, USA

    Hamdy M. A. Ahmed

  3. Rheumatology and Clinical immunology, Faculty Of Medicine Cairo University, Giza, Egypt

    Hamdy M. A. Ahmed

  4. Internal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt

    Gaafar Ragab

Authors
  1. Mervat Mattar
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Hamdy M. A. Ahmed
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Gaafar Ragab
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Saskatoon Cancer Centre College of Medicine University of Saskatchewan, Saskatoon, SK, Canada

    Hadi Goubran

  2. Faculty of Medicine Cairo University, Giza, Egypt

    Gaafar Ragab

  3. College of Human Medicine Michigan State University, East Lansing, MI, USA

    Suzy Hassouna

Rights and permissions

Reprints and permissions

Copyright information

© 2020 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Mattar, M., Ahmed, H.M.A., Ragab, G. (2020). Antiphospholipid Syndrome. In: Goubran, H., Ragab, G., Hassouna, S. (eds) Precision Anticoagulation Medicine. Springer, Cham. https://doi.org/10.1007/978-3-030-25782-8_8

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-25782-8_8

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-25781-1

  • Online ISBN: 978-3-030-25782-8

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation