Antiphospholipid syndrome

Czech version

Authors: Z. Fojtík
Authors‘ workplace: Interní hematoonkologická klinika Lékařské fakulty MU a FN Brno, pracoviště Bohunice, přednosta prof. MUDr. Jiří Vorlíček, CSc.
Published in: Vnitř Lék 2006; 52(7-8): 718-725
Category: 130th Internal Medicine Day - Rheumatology in clinical practice

Overview

Antiphospholipid syndrome (APS), first described and termed in the early 1980’s, is defined by its clinical and laboratory signs. If other disorders inducing antibody production are excluded, APS may be considered a primary disease. Or it can be a secondary condition that is usually seen in systemic diseases of connective tissues. The main concept of APS is based on strong association between its clinical and laboratory manifestations and patient's medical history. Clinical signs include recurrent arterial and venous thrombosis or repeated fetal losses; laboratory criteria involve lupus anticoagulant (LA) syndrome or medium and high titres of anticardiolipin (aCL) antibodies. Thus defined, APS represents the most common form of acquired thrombophilic conditions while the basic concept lies in strong association between specific clinical manifestations and repeated detection of LA or aCL. Antiphospholipid antibodies (LA and aCL) are extremely heterogeneous antibody groups whose effect on processes at different levels of coagulation cascade varies. Their effect can be analogical to the action of phospholipid surfaces at various points of blood coagulation. This process usually results in development of a certain degree of thrombophilia. Present level of understanding of APS indicates that characteristics of clinical signs and detection and evaluation of antiphospholipid antibodies have not been definitely established yet and are to be specified with the help of molecular geneticists, immunologists, biochemists, hematologists and rheumatologists.

Key words:
antiphospholipid syndrome – lupus anticoagulant – anticardiolipin antibodies

Sources

1. Asherson RA, Cervera R, de Groot PG et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12: 530-534.

2. Bertolaccini ML, Khamashta MA, Hughes GR. Diagnosis of antiphospholipid syndrome. Rheumatology 2005; 1: 40-46.

3. Bevers EM, Galli M, Barbui T et al. Lupus anticoagulant IgGs (LA) are not directed to phospholipids only, but to a complex of lipid-bound human prothrombin. Thromb Haemost 1991; 66: 629-632.

4. Cervera R, Oiette JC, Font J et al. Antiphospholipid syndrome. Clinical and Immunological manifestation and patterns of disease expression in a cohort of 1 000 patients. Arthritis Rheumatism 2002; 46: 1019-1027.

5. Crowther MA, Gingsberg JS, Julian J et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med 2003; 349: 1133-1138.

6. Derksen RH, de Groot PG. Clinical consequences of antiphospholipid antibodies. Neth J Med 2004; 62: 273-278.

7. Derksen RH, Khamashta MA, Branch DW. Management of the obstetric antiphospholipid syndrome. Arthritis Rheumatism 2004; 50: 1028-1039.

8. Derksen RH, de Groot PG, Kater L et al. Patients with antiphospholipid antibodies and venous thrombosis should receive long-term anticoagulant treatment. Ann Rheum Dis 1993; 52: 689-692.

9. Derksen RH, de Groot PG, Kappelle LJ. Low dose aspirin after ischemic stroke associated with antiphospholipid syndrome. Neurology 2003; 61: 111-114.

10. DeGroot PG, Derksen RW. Antiphospholipid antibodies: update on detection, pathophysiology, and treatment. Curr Opin Hematol 2004; 11: 165-169.

11. Dostál C. Antifosfolipidový syndrom. In: Systémový lupus erythematodes. Cheb Medprint 1997: 136-147.

12. Erkan D, Ortel TL, Lockshin MD. Warfarin in antiphospholipid syndrome - time to explore new horizons. J Rheumatol 2005; 32: 208-212.

13. Farquharson RG, Quenby S, Greaves M. Antiphospholipid syndrom in pregnancy: a randomized, controlled trial of treatment. Obstet Gynecol 2002; 100: 408-413.

14. Finazzi G, Marchioli R, Brancaccio V et al. A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome. J Thromb Haemost 2005; 3: 848-853.

15. Fojtík Z, Beránek M, Klabusay M et al. Výskyt vybraných antifosfolipidových protilátek u skupiny nemocných se systémovým lupus erythematodes. Vnitř Lék 2004; 50: 267-273.

16. Galli M. Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood 2003; 101: 1827-1832.

17. Godfrey T, Cruz D. Antiphospholipid syndrome: general features. In: Hughes syndrome, Antiphospholipid syndrome. London Springer-Verlag 2000: 8-19.

18. Harris EN, Baguley E, Asherson RA et al. Clinical and serological features of the antiphospholipid syndrome. (Abstract). Br J Rhematol 1987; 26: 19.

19. Hughes GRV. The anticardiolipin syndrome. Clin Exp Rheumatol 1985; 3: 285.

20. Kearon C, Gent M, Hirsh J et al. A comparison of three months of anticoagulation with extended anticoagulation for a first episode of idiopathic venous thromboembolism. N Engl J Med 1999; 340: 901-907.

21. Khamastha MA, Cuadrado MJ, Mujic F et al. The management of thrombosis in the antiphospholid-antibody syndrome. N Engl J Med 1995; 332: 993-997.

22. Kutteh WH, Ermel LD. A clinical trial for the treatment of antiphospholipid antibody-associated recurrent pregnancy loss with lower dose heparin and aspirin. Am J Reprod Immunol 1996; 35: 402-407.

23. Levine SR, Brey RL, Tilley BC et al. Antiphospholipid antibodies and subsequent thrombo-occlusive events in patients with ischemic stroke. JAMA 2004; 291: 576-584.

24. Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. N Engl J Med 2002; 346: 752-763.

25. Merkel PA, Chang Y, Pierangeli SS et al. The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue disease. Am J Med 1996; 101: 576-583.

26. Moll S, Ortel TL. Monitoring warfarin therapy in patients with lupus anticoagulants. Ann Intern Med 1997; 127: 177-185.

27. Petri M. Thrombosis and systemic lupus erythematosus: the Hopkins lupus cohort perspective. Scand J Rheum 1996; 191-193.

28. Piette JC. 1996 diagnostic and classification criteria for the antiphospholipid/cofactors: a missin impossible? Lupus 1996; 5: 354-363.

29. Rai R, Cohen H, Dave M et al. Randomised controlled trial of aspirin and aspirin plus heparin in pregnant women with recurrent miscarriage associated with phospholipid antibodies (or antiphospholipid antibodies). Brit Med J 1997; 314: 253-257.

30. Rosove MH, Brewer PM. Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients: Ann Intern Med 1992; 117: 303-308.

31. Ruiz-Irastorza G, Khamashta MA, Hunt BJ et al. Bleeding and recurrent thrombosis in definite antiphospholipid syndrome: analysis of a series of 66 patients treated with oral anticoagulation to a target international normalized ratio of 3,5. Arch Intern Med 2002; 162: 1164-1169.

32. Sachse C, Luethke K, Hartung K et al. Significance of antibodies to cardiolipn in unselected patients with systemic lupus erythematosus: clinical and laboratory association. Rheumatol Int 1995; 15: 23-29.

33. Schulman S, Svenungsson E, Granqvist S. Anticardiolipin antibodies predict early recurrence of thromboembolism and death among patients with venous thromboembolism following anticoagulant therapy. Duration of Anticoagulation Study Group. Am J Med 1998; 104: 332-338.

34. The APASS Investigators. Antiphospholipid antibodies and subsequent thrombo-occlusive events in patients with ischemic stroke. JAMA 2004; 291: 576-584.

35. Vianna JL, Khamashta MA, 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.

36. Wilson WA, Gharavi AE, Koike T et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome. Arthritis Rheum 1999; 42: 1309-1311.