Skip to main content

Advertisement

Log in

The Laboratory Diagnosis of the Antiphospholipid Syndrome

  • Review Article
  • Published:
Indian Journal of Hematology and Blood Transfusion Aims and scope Submit manuscript

Abstract

The Antiphospholipid Syndrome (APS) is classified based on the presence of both clinical and laboratory criteria. Both sets of criteria are subject to much review and intense research as it is becoming increasingly clear that no single test is specific for defining this autoimmune disorder. A number of leading international bodies have released guidelines in an attempt to improve the laboratory testing and reporting. The current review is an appraisal of some of the literature pertaining to the laboratory testing.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Khamashta M, Hughes GRV (1999) Hughes syndrome: a short history of the antiphospholipid syndrome. In: Schoenfeld Y (ed) The decade of autoimmunity. Elsevier Science BV

  2. Nayfe R, Uthman I, Aoun J, SaadAldin E, Merashli M, Khamashta MA (2013) Seronegative antiphospholipid syndrome. Rheumatology (Oxf Engl) 52:1358–1367

    Article  Google Scholar 

  3. Meroni PL, Chighizola CB, Rovelli F, Gerosa M (2014) Antiphospholipid syndrome in 2014: more clinical manifestations, novel pathogenic players and emerging biomarkers. Arthritis Res Ther 16:1–14

    Article  Google Scholar 

  4. Forastiero R (2012) Bleeding in the antiphospholipid syndrome. Hematology (Amst Neth) 17(Suppl 1):153–155

    Google Scholar 

  5. de Groot PG, Urbanus RT, Derksen RHWM (2012) Pathophysiology of thrombotic APS: Where do we stand? Lupus 21:704–707

    Article  PubMed  Google Scholar 

  6. Giannakopoulos B, Krilis SA (2013) The pathogenesis of the antiphospholipid syndrome. N Engl J Med 368:1033–1044

    Article  CAS  PubMed  Google Scholar 

  7. de Groot PG, Meijers JCM (2011) β(2)–Glycoprotein I: evolution, structure and function. J Thromb Haemost JTH 9:1275–1284

    Article  PubMed  Google Scholar 

  8. Krilis SA, Giannakopoulos B (2014) Laboratory methods to detect antiphospholipid antibodies. Hematol Am Soc Hematol Educ Program 2014:321–328

    Google Scholar 

  9. de Groot PG, Urbanus RT (2012) The significance of autoantibodies against β2-glycoprotein I. Blood 120:266–274

    Article  PubMed  Google Scholar 

  10. Lackner KJ, Müller-Calleja N (2016) Pathogenesis of the antiphospholipid syndrome revisited: time to challenge the dogma. J Thromb Haemost JTH 14:1117–1120

    Article  CAS  PubMed  Google Scholar 

  11. Wilson WA, Gharavi AE, Koike T, Lockshin MD, Branch DW, Piette JC et al (1999) International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 42:1309–1311

    Article  CAS  PubMed  Google Scholar 

  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 JTH 4:295–306

    Article  CAS  PubMed  Google Scholar 

  13. Cousins L, Pericleous C, Khamashta M, Bertolaccini ML, Ioannou Y, Giles I et al (2015) Antibodies to domain I of β-2-glycoprotein I and IgA antiphospholipid antibodies in patients with “seronegative” antiphospholipid syndrome. Ann Rheum Dis 74:317–319

    Article  CAS  PubMed  Google Scholar 

  14. Pengo V, Testa S, Martinelli I, Ghirarduzzi A, Legnani C, Gresele P et al (2015) Incidence of a first thromboembolic event in carriers of isolated lupus anticoagulant. Thromb Res 135:46–49

    Article  CAS  PubMed  Google Scholar 

  15. Pengo V, Ruffatti A, Legnani C, Testa S, Fierro T, Marongiu F et al (2011) Incidence of a first thromboembolic event in asymptomatic carriers of high-risk antiphospholipid antibody profile: a multicenter prospective study. Blood 118:4714–4718

    Article  CAS  PubMed  Google Scholar 

  16. Mustonen P, Lehtonen KV, Javela K, Puurunen M (2014) Persistent antiphospholipid antibody (aPL) in asymptomatic carriers as a risk factor for future thrombotic events: a nationwide prospective study. Lupus 23:1468–1476

    Article  CAS  PubMed  Google Scholar 

  17. de Groot PG, Lutters B, Derksen RHWM, Lisman T, Meijers JCM, Rosendaal FR (2005) Lupus anticoagulants and the risk of a first episode of deep venous thrombosis. J Thromb Haemost JTH 3:1993–1997

    Article  PubMed  Google Scholar 

  18. de Laat B, Derksen RHWM, Urbanus RT, de Groot PG (2005) IgG antibodies that recognize epitope Gly40-Arg43 in domain I of beta 2-glycoprotein I cause LAC, and their presence correlates strongly with thrombosis. Blood 105:1540–1545

    Article  PubMed  Google Scholar 

  19. Banzato A, Pozzi N, Frasson R, De Filippis V, Ruffatti A, Bison E et al (2011) Antibodies to domain I of β(2)Glycoprotein I are in close relation to patients risk categories in antiphospholipid syndrome (APS). Thromb Res 128:583–586

    Article  CAS  PubMed  Google Scholar 

  20. Pengo V, Banzato A, Denas G, Jose SP, Bison E, Hoxha A et al (2013) Correct laboratory approach to APS diagnosis and monitoring. Autoimmun Rev 12:832–834

    Article  CAS  PubMed  Google Scholar 

  21. Forastiero R (2014) Multiple antiphospholipid antibodies positivity and antiphospholipid syndrome criteria re-evaluation. Lupus 23:1252–1254

    Article  CAS  PubMed  Google Scholar 

  22. Park SH, Jang S, Park C-J, Chi H-S (2016) Clinical application of revised laboratory classification criteria for antiphospholipid antibody syndrome: Is the follow-up interval of 12 weeks instead of 6 weeks significantly useful? BioMed Res Int 2016:2641526

    PubMed  PubMed Central  Google Scholar 

  23. Pengo V, Denas G, Padayattil SJ, Zoppellaro G, Bison E, Banzato A et al (2015) Diagnosis and therapy of antiphospholipid syndrome. Pol Arch Med Wewn 125:672–677

    PubMed  Google Scholar 

  24. Pengo V, Tripodi A, Reber G, Rand JH, Ortel TL, Galli M et al (2009) Update of the guidelines for lupus anticoagulant detection. Subcommittee on Lupus anticoagulant/antiphospholipid antibody of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis. J Thromb Haemost JTH 7:1737–1740

    Article  CAS  PubMed  Google Scholar 

  25. Rand JH, Wolgast LR (2012) Dos and don’ts in diagnosing antiphospholipid syndrome. Hematol Am Soc Hematol Educ Program 2012:455–459

    Google Scholar 

  26. Devreese KMJ, Pierangeli SS, de Laat B, Tripodi A, Atsumi T, Ortel TL et al (2014) Testing for antiphospholipid antibodies with solid phase assays: guidance from the SSC of the ISTH. J Thromb Haemost 12:792–795

    Article  CAS  PubMed  Google Scholar 

  27. Keeling D, Mackie I, Moore GW, Greer IA, Greaves M (2012) British Committee for Standards in haematology. Guidelines on the investigation and management of antiphospholipid syndrome. Br J Haematol 157:47–58

    Article  CAS  PubMed  Google Scholar 

  28. Moore GW (2014) Recent guidelines and recommendations for laboratory detection of lupus anticoagulants. Semin Thromb Hemost 40(2):163–171

    Article  CAS  PubMed  Google Scholar 

  29. De Craemer AS, Musial J, Devreese KM (2016) Role of anti-domain 1-β2 glycoprotein I antibodies in the diagnosis and risk stratification of antiphospholipid syndrome. J Thromb Haemost 14(9):1779–1787. doi:10.1111/jth.13389

    Article  PubMed  Google Scholar 

  30. Simmons DP, Herskovits AZ, Battinelli EM, Schur PH, Lemire SJ, Dorfman DM (2016) Lupus anticoagulant testing using two parallel methods detects additional cases and predicts persistent positivity. Clin Chem Lab Med. doi:10.1515/cclm-2015-0790. pii: /j/cclm.ahead-of-print/cclm-2015-0790/cclm-2015-0790.xml

    PubMed  Google Scholar 

  31. Ahluwalia J, Sreedharanunni S, Kumar N, Masih J, Bose SK, Varma N et al (2016) Thrombotic primary antiphospholipid syndrome: the profile of antibody positivity in patients from North India. Int J Rheum Dis 19(9):903–912

    Article  CAS  PubMed  Google Scholar 

  32. Meneghel L, Ruffatti A, Gavasso S, Tonello M, Mattia E, Spiezia L et al (2015) The clinical performance of a chemiluminescent immunoassay in detecting anti-cardiolipin and anti-β2 glycoprotein I antibodies. A comparison with a homemade ELISA method. Clin Chem Lab Med 53:1083–1089

    Article  CAS  PubMed  Google Scholar 

  33. Oku K, Amengual O, Kato M, Bohgaki T, Horita T, Yasuda S et al (2016) Significance of fully automated tests for the diagnosis of antiphospholipid syndrome. Thromb Res 146:1–6

    Article  CAS  PubMed  Google Scholar 

  34. Sciascia S, Sanna G, Murru V, Roccatello D, Khamashta MA, Bertolaccini ML (2015) The global anti-phospholipid syndrome score in primary APS. Rheumatology (Oxf Engl) 54:134–138

    Article  Google Scholar 

  35. Sciascia S, Sanna G, Murru V, Roccatello D, Khamashta MA, Bertolaccini ML (2013) GAPSS: the Global Anti-Phospholipid Syndrome Score. Rheumatology (Oxf Engl) 52:1397–1403

    Article  Google Scholar 

  36. Sciascia S, Cuadrado MJ, Sanna G, Murru V, Roccatello D, Khamashta MA et al (2014) Thrombotic risk assessment in systemic lupus erythematosus: validation of the global antiphospholipid syndrome score in a prospective cohort. Arthritis Care Res 66:1915–1920

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Jasmina Ahluwalia.

Ethics declarations

Conflict of interest

Dr Jasmina Ahluwalia and Dr Sreejesh Sreedharanunni have no conflict of interest to declare.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors. This article does not contain any studies with animals performed by any of the authors.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Ahluwalia, J., Sreedharanunni, S. The Laboratory Diagnosis of the Antiphospholipid Syndrome. Indian J Hematol Blood Transfus 33, 8–14 (2017). https://doi.org/10.1007/s12288-016-0739-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12288-016-0739-y

Keywords

Navigation