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Semin Thromb Hemost 2008; 34(3): 219-226
DOI: 10.1055/s-0028-1082265
© Thieme Medical Publishers

Primary, Secondary, and Catastrophic Antiphospholipid Syndrome: What's in a Name?

E. Nigel Harris1 , Silvia S. Pierangeli2
  • 1The University of the West Indies, Kingston, Jamaica
  • 2Department of Medicine, Division of Rheumatology, University of Texas at Galveston, Galveston, Texas
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Publikationsdatum:
21. August 2008 (online)

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ABSTRACT

The association of the lupus anticoagulant with thrombosis and recurrent pregnancy loss was first recognized over a 20-year period between the early 1960s and early 1980s. The introduction of the anticardiolipin test in 1983 and the recognition of its association with clinical features similar to the lupus anticoagulant led to an exponential growth of interest in this disorder. The belief that anticardiolipin antibodies and lupus anticoagulant belonged to the family of antiphospholipid antibodies led to the disorder being named the antiphospholipid syndrome (APS). Efforts by individual investigators to introduce criteria for classification of APS and to standardize anticardiolipin antibody and lupus anticoagulant tests were started in the mid-1980s to ensure more reliable recognition and treatment of affected patients. Another layer of complexity was introduced with recognition that many anticardiolipin antibody–positive sera also bound the antigen β2 glycoprotein I. With increasingly sophisticated epidemiologic and prospective studies in the 1990s, more structured and better-documented criteria for APS were introduced in 1999 and modified in 2006. These criteria have been widely adopted. Whereas data supporting subclassification of APS into primary and secondary subgroups remain tenuous, a small percentage of patients do appear subject to clinical features termed the catastrophic antiphospholipid syndrome. Introduction of classification criteria for APS has enabled more reliable prospective studies, the promise of better management, and more valid tests for recognition of the disorder.

KEYWORDS

Antiphospholipid syndrome - lupus anticoagulant - anticardiolipin antibody test - antiphospholipid antibodies - anti–β2 glycoprotein I

REFERENCES

  • 1 Conley C L, Hartmann R C. A hemorrhagic disorder caused by circulating anticoagulant in patients with disseminated lupus erythematosus.  J Clin Invest. 1952;  31 621-622
    PubMedGoogle Scholar
  • 2 Lechner K. Acquired inhibitors in auto and isoimmune diseases.  Haemostasis. 1974;  3 65-93
    PubMedGoogle Scholar
  • 3 Feinstein D I, Rapaport S I. Acquired inhibitors of blood coagulation. In Spaet TN Progress in Hemostasis and Thrombosis, 1st ed. New York, NY; Grune and Stratton 1982: 75-95
    Google Scholar
  • 4 Shapiro S S, Thiagarajan P. Lupus anticoagulant.  Prog Hemost Thromb. 1982;  6 263-285
    PubMedGoogle Scholar
  • 5 Bowie E JW, Thompson J H, Pascuzzi C A, Owen C A. Thrombosis in systemic lupus erythematosus despite circulating anticoagulants.  J Lab Clin Med. 1963;  62 416-430
    PubMedGoogle Scholar
  • 6 Soulier J P, Boffa M C. Avortements à répétition, thromboses, et anticoagulant circulant anti-thromoboplastine.  Nouv Presse Med. 1980;  9 859-864
    PubMedGoogle Scholar
  • 7 Carreras L O, Vermylen J G. “Lupus” anticoagulant and thrombosis – possible role of inhibition of prostacyclin formation.  Thromb Haemost. 1982;  48 38-40
    PubMedGoogle Scholar
  • 8 Boey M L, Colaco C B, Gharavi A E et al.. Thrombosis in systemic lupus erythematosus: striking association with the presence of circulating anticoagulant.  BMJ. 1983;  287 1021-1023
    CrossrefPubMedGoogle Scholar
  • 9 Nilsson I M, Asstedt B, Hedner U et al.. Intrauterine death and circulating anticoagulant “antithromboplastin”.  Acta Med Scand. 1975;  197 153-159
    PubMedGoogle Scholar
  • 10 Mueh J R, Herbst K D, Rapaport S I. Thrombosis in patients with the lupus anticoagulant.  Ann Intern Med. 1980;  92 156-159
    PubMedGoogle Scholar
  • 11 Hughes G RV. The Prosser-White oration 1983. Connective tissue disease and the skin.  Clin Exp Dermatol. 1984;  9 535-544
    CrossrefPubMedGoogle Scholar
  • 12 Harris E N, Gharavi A E, Boey M L et al.. Anticardiolipin antibodies: detection by radioimmunoassay and association with thrombosis in systemic lupus erythematosus.  Lancet. 1983;  2 1211-1214
    PubMedGoogle Scholar
  • 13 Harris E N, Gharavi A E, Loizou S et al.. Cross-reactivity of antiphospholipid antibodies.  J Clin Lab Immunol. 1985;  16 1-6
    PubMedGoogle Scholar
  • 14 Harris E N, Gharavi A E, Hughes G RV. Anti-phospholipid antibodies.  Clin Rheum Dis. 1985;  11 591-606
    PubMedGoogle Scholar
  • 15 Lubbe W F, Palmer S J, Butler W S, Laggins G C. Fetal survival after prednisone suppression of maternal lupus anticoagulant.  Lancet. 1983;  i 1361-1363
    PubMedGoogle Scholar
  • 16 Asherson R A, Chan J KH, Harris E N et al.. Anticardiolipin antibody, recurrent thrombosis and warfarin withdrawal.  Ann Rheum Dis. 1985;  44 823-825
    CrossrefPubMedGoogle Scholar
  • 17 Harris E N, Chan J KH, Asherson R A et al.. Thrombosis, recurrent fetal loss and thrombocytopemia: predictive value of the anticardiolipin test.  Arch Intern Med. 1986;  146 2153-2156
    CrossrefPubMedGoogle Scholar
  • 18 Harris E N, Gharavi A E, Patel S P, Hughes G RV. Evaluation of the anti-cardiolipin antibody test: report of an international workshop held 4 April 1986.  Clin Exp Immunol. 1987;  68 215-222
    PubMedGoogle Scholar
  • 19 Hughes G RV, Harris E N, Gharavi A E. The anticardiolipin syndrome.  J Rheumatol. 1986;  13 486-489
    PubMedGoogle Scholar
  • 20 Harris E N. Syndrome of the Black Swan.  Br J Rheumatol. 1987;  26 324-326
    CrossrefPubMedGoogle Scholar
  • 21 Asherson R A. A primary anti-phospholipid syndrome?.  J Rheumatol. 1988;  15 1742-1745
    PubMedGoogle Scholar
  • 22 Alarcon Segovia D, Sanchez–Guerrero J. Primary antiphospholipid syndrome.  J Rheumatol. 1989;  16 482-487
    PubMedGoogle Scholar
  • 23 Asherson R A. The catastrophic antiphospholipid antibody syndrome.  J Rheumatol. 1992;  19 508-512
    PubMedGoogle Scholar
  • 24 Greisman S G, Thaya Paran R S, Godwin T A, Lockshin M D. Occlusive vasculopathy in systemic lupus erythematosus – association with anticardiolipin antibody.  Arch Intern Med. 1991;  151 389-392
    CrossrefPubMedGoogle Scholar
  • 25 Harris E N, Bos K. An acute disseminated coagulopathy – vasculopathy associated with the antiphospholipid syndrome.  Arch Intern Med. 1991;  151 231-232
    CrossrefPubMedGoogle Scholar
  • 26 McNeil H P, Simpson J, Chesterman C N, Krilis S A. Antiphospholipid antibodies are directed against a complex antigen that includes lipid binding inhibitor of coagulation: beta-2-glycoprotein I (apolipoprotein II).  Proc Natl Acad Sci U S A. 1990;  87 4120-4124
    CrossrefPubMedGoogle Scholar
  • 27 Galli M, Comfurius P, Maassen C et al.. Anticardiolipin antibodies directed not to cardiolipin but to a plasma protein cofactor.  Lancet. 1990;  355 1544-1547
    PubMedGoogle Scholar
  • 28 Matsuura E, Igarashi Y, Yasuda T et al.. Anticardiolipin antibodies recognize β2 glycoprotein 1 structure altered by interacting with an oxygen modified solid phase surface.  J Exp Med. 1994;  179 457-462
    CrossrefPubMedGoogle Scholar
  • 29 Galli M, Lucian D, Bertolini G, Barbui J. Anti-beta2-glycoprotein 1, anti-prothrombin antibodies and the risk of thrombosis on the antiphospholipid syndrome.  Blood. 2003;  102 2717-2723
    CrossrefPubMedGoogle Scholar
  • 30 Reber G, deMoerloose P. Anti-beta2-glycoprotein 1 antibodies – when and how they should be measured?.  Thromb Res. 2004;  114 527-531
    CrossrefPubMedGoogle Scholar
  • 31 Faden D, Tincani A, Tanzi P et al.. Anti-beta2 glycoprotein 1 antibodies in a general obstetric population: preliminary results on the prevalence and correlation with pregnancy outcome. Anti-beta2 glycoprotein 1 antibodies are associated with some obstetrical complications, mainly preeclampsia-eclampsia.  Eur J Obstet Gynecol Reprod Biol. 1997;  73 37-42
    CrossrefPubMedGoogle Scholar
  • 32 Miyakis S, Lockshin M D, Atsumi T et al.. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome.  J Thromb Haemost. 2006;  4 295-306
    CrossrefPubMedGoogle Scholar
  • 33 Lackner K J, Peetz D, Von Landenberg P. Revision of the Sapporo criteria for the antiphospholipid syndrome – coming to grips with evidence and Thomas Bayes?.  Thromb Haemost. 2006;  95 917-919
    PubMedGoogle Scholar
  • 34 Nash M J, Camilleri R S, Kunka S. The anticardiolipin assay is required for sensitive screening for antiphospholipid antibodies.  J Thromb Haemost. 2004;  2 1077-1081
    CrossrefPubMedGoogle Scholar
  • 35 Wilson W A, Gharavi A E, Koike T et al.. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome.  Arthritis Rheum. 1999;  42 1309-1311
    CrossrefPubMedGoogle Scholar
  • 36 Escalante A, Brey R L, Mitchell B D, Dreiner U. Accuracy of anticardiolipin antibodies in identifying a history of thrombosis among patients with systemic lupus erythematosus.  Am J Med. 1995;  98 559-565
    CrossrefPubMedGoogle Scholar
  • 37 Levine S R, Salowich-Palm L, Sawaya K L et al.. IgG anticardiolipin antibody titer > 40 GPL and the risk of subsequent thrombo-occlusive events and death. A prospective cohort study.  Stroke. 1997;  28 1660-1665
    CrossrefPubMedGoogle Scholar
  • 38 Alarcon-Segovia D, Perez-Vazquez M E, Villa A R et al.. Preliminary classification criteria for the antiphospholipid syndrome within systemic lupus erythematosus.  Semin Arthritis Rheum. 1992;  21 275-286
    CrossrefPubMedGoogle Scholar
  • 39 Lockshin M D, Sammaritano L R, Schwartzman S. Validation of the Sapporo criteria for antiphospholipid syndrome.  Arthritis Rheum. 2000;  43 440-443
    CrossrefPubMedGoogle Scholar
  • 40 Vianna J L, Khamashta M A, Ordi-Ros J et al.. Comparison of the primary and secondary antiphospholipid syndrome: a European Multicenter Study of 114 patients.  Am J Med. 1994;  96 3-9
    CrossrefPubMedGoogle Scholar
  • 41 Asherson R A, Cervera R, deGroot P G et al.. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines.  Lupus. 2003;  12 530-534
    CrossrefPubMedGoogle Scholar
  • 42 Tan E M, Cohen A S, Fries J F et al.. Revised criteria for the classification of system lupus erythematosus.  Arthritis Rheum. 1982;  25 1271-1277
    CrossrefPubMedGoogle Scholar
  • 43 The APASS Investigators . Antiphospholipid antibodies and subsequent thrombo-occlusive events in patients with ischemic stroke.  JAMA. 2004;  291 576-584
    CrossrefPubMedGoogle Scholar
  • 44 Branch D W, Khamashta M A. Antiphospholipid syndrome: obstetric diagnosis, management, and controversies.  Obstet Gynecol. 2003;  101 1333-1344
    CrossrefPubMedGoogle Scholar
  • 45 Harris E N. The Second International Anti-cardiolipin Standardization workshop/the Kingston Anti-phospholipid Antibody Study (KAPS) group.  Am J Clin Pathol. 1990;  94 476-484
    PubMedGoogle Scholar
  • 46 Harris E N, Pierangeli S S. Revisiting the anticardiolipin test and its standardization.  Lupus. 2002;  11 269-276
    CrossrefPubMedGoogle Scholar
  • 47 Tincani A, Allegri F, Sanmarco M et al.. Anticardiolipin antibody assay: a methodological analysis for a better consensus in routine determinations – a cooperative project of the European Antophospholipid Forum.  Thromb Haemost. 2001;  86 575-583
    PubMedGoogle Scholar
  • 48 Wong R CW, Gillis D, Adelstein S et al.. Consensus guidelines on anti-cardiolipin antibody testing and reporting.  Pathology. 2004;  36 63-68
    CrossrefPubMedGoogle Scholar
  • 49 Favaloro E J, Silvestrini R, Mohammed A. Clinical utility of anticardiolipin antibody assays: high inter-laboratory variation and limited consensus by participants of external quality assurance programs signals a cautious approach.  Pathology. 1999;  31 142-147
    CrossrefPubMedGoogle Scholar
  • 50 Pierangeli S S, Harris E N. A quarter of a century in anticardiolipin antibody testing and attempted standardization has led us to here, which is?.  Semin Thromb Hemost. 2008;  34 , (in press)
    PubMedGoogle Scholar
  • 51 Lopez-Soto A, Cervera R, Font J et al.. Isotype distribution and clinical significance of antibodies to cardiolipin, phosphatidic acid phosphatidyl inositol and phosphatidyl serine in systemic lupus erythematosus: prosective analysis in a series of 92 patients.  Clin Exp Rheumatol. 1997;  15 143-149
    PubMedGoogle Scholar
  • 52 Branch D W, Silver R, Pierangeli S et al.. Antiphospholipid antibodies other than lupus anticoagulant and anticardiolipin antibodies in women with recurrent pregnancy loss, fertile controls and antiphospholipid syndrome.  Obstet Gynecol. 1997;  89 549-555
    CrossrefPubMedGoogle Scholar
  • 53 Merkel P A, Chang Y, Pierangeli S S, Harris E N, Polisson R P. Comparison between the standard anticardiolipin antibody assay and a new phospholipid assay in patients with a variety of connective tissue diseases.  J Rheumatol. 1999;  26 591-596
    PubMedGoogle Scholar
  • 54 Atsumi T, Ieko M, Bertolccini M et al.. Association of autoantibodies against the phosphatidyl serine-prothrobim complex with manifestations of the antiphospholipid syndrome and with the presence of lupus anticoagulant.  Arthritis Rheum. 2000;  43 1982-1993
    CrossrefPubMedGoogle Scholar

E. Nigel HarrisM.D. 

Vice Chancellor, The University of the West Indies

Mona, Kingston 7, Jamaica W.I

eMail: vcoffice@uwimona.edu.jm