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Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients
  1. R Cervera1,
  2. M A Khamashta2,
  3. Y Shoenfeld3,
  4. M T Camps4,
  5. S Jacobsen5,
  6. E Kiss6,
  7. M M Zeher6,
  8. A Tincani7,
  9. I Kontopoulou-Griva8,
  10. M Galeazzi9,
  11. F Bellisai9,
  12. P L Meroni10,
  13. R H W M Derksen11,
  14. P G de Groot12,
  15. E Gromnica-Ihle13,
  16. M Baleva14,
  17. M Mosca15,
  18. S Bombardieri15,
  19. F Houssiau16,
  20. J-C Gris17,
  21. I Quéré17,
  22. E Hachulla18,
  23. C Vasconcelos19,
  24. B Roch20,
  25. A Fernández-Nebro21,
  26. J-C Piette22,
  27. G Espinosa1,
  28. S Bucciarelli1,
  29. C N Pisoni2,
  30. M L Bertolaccini2,
  31. M-C Boffa22,
  32. G R V Hughes2
  1. 1
    Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain
  2. 2
    Lupus Unit, Rayne Institute, St Thomas’ Hospital, London, UK
  3. 3
    Research Centre for Autoimmune Diseases, Chaim-Sheba Medical Centre, Tel-Hashomer, Israel
  4. 4
    Unidad de Enfermedades Autoinmunitarias Sistémicas, Servicio de Medicina Interna, Hospital Regional “Carlos Haya”, Málaga, Spain
  5. 5
    Department of Rheumatology, Copenhagen University Hospital at Rigshospitalet, Copenhagen, Denmark
  6. 6
    3rd Department of Medicine, University of Debrecen, Medical and Health Science Centre, Debrecen, Hungary
  7. 7
    Servizio di Immunologia Clinica e Allergologia, Spedali Civili, Azienda Ospedaliera, Brescia, Italy
  8. 8
    Transfusion and Haemophilia Centre, Hippocration Hospital, Athens, Greece
  9. 9
    Istituto di Reumatologia, Policlinico “Le Scotte”, Siena, Italy
  10. 10
    Allergy and Clinical Immunology Unit, Dipartimento di Medicina Interna, IRCCS Istituto Auxologico, Università di Milano, Milan, Italy
  11. 11
    Department of Rheumatology and Clinical Immunology, University Medical Centre, Utrecht, The Netherlands
  12. 12
    Department of Haematology, Laboratory of Thrombosis and Haemostasis, University Medical Centre, Utrecht, The Netherlands
  13. 13
    Rheumaklinik Berlin-Buch, Immanuel-Krankenhaus GmbH, Berlin, Germany
  14. 14
    Laboratory of Clinical Immunology, Clinical Centre of Allergology, Medical University, Sofia, Bulgaria
  15. 15
    Dipartimento di Medicina Interna, Università di Pisa, Pisa, Italy
  16. 16
    Service de Rhumatologie, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
  17. 17
    Laboratoire dHématologie, CHU, Nìmes, France
  18. 18
    Service de Médecine Interne, Hôpital Claude Huriez, Lille, France
  19. 19
    Departamento de Medicina Interna, Hospital Geral San António, Porto, Portugal
  20. 20
    Rheumatologische Ambulanz, Medizinische Klinik III und Medizinische Poliklinik, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
  21. 21
    Sección de Reumatología, Hospital Clínico Universitario, Málaga, Spain
  22. 22
    Department of Internal Medicine, Hôpital Pitié-Salpêtrière, Paris, France
  1. Correspondence to Dr R Cervera, Servei de Malalties Autoimmunes, Hospital Clínic, Villarroel 170, 08036-Barcelona, Catalonia, Spain; rcervera{at}


Objectives: To identify the main causes of morbidity and mortality in patients with antiphospholipid syndrome (APS) during a 5-year period and to determine clinical and immunological parameters with prognostic significance.

Methods: The clinical and immunological features of a cohort of 1000 patients with APS from 13 European countries who had been followed up from 1999 to 2004 were analysed.

Results: 200 (20%) patients developed APS-related manifestations during the 5-year study period. Recurrent thrombotic events appeared in 166 (16.6%) patients and the most common were strokes (2.4% of the total cohort), transient ischaemic attacks (2.3%), deep vein thromboses (2.1%) and pulmonary embolism (2.1%). When the thrombotic events occurred, 90 patients were receiving oral anticoagulants and 49 were using aspirin. 31/420 (7.4%) patients receiving oral anticoagulants presented with haemorrhage. 3/121 (2.5%) women with only obstetric APS manifestations at the start of the study developed a new thrombotic event. A total of 77 women (9.4% of the female patients) had one or more pregnancies and 63 (81.8% of pregnant patients) had one or more live births. The most common fetal complications were early pregnancy loss (17.1% of pregnancies) and premature birth (35% of live births). 53 (5.3% of the total cohort) patients died. The most common causes of death were bacterial infection (21% of deaths), myocardial infarction (19%) and stroke (13%). No clinical or immunological predictor of thrombotic events, pregnancy morbidity or mortality was detected.

Conclusion: Patients with APS still develop significant morbidity and mortality despite current treatment (oral anticoagulants or antiaggregants, or both).

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  • For numbered affiliations see end of article

  • Josep Font (a member of the Euro-Phospholipid Project Group) died during the preparation of this manuscript and the authors want to dedicate this article to his memory.

  • A complete list of members of the Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies) is given in Appendix A.

  • Funding Supported in part by grants FISS 2003/028 from Fondo de Investigaciones Sanitarias of Spain, Ricerca Corrente 2000 from IRCCS Istituto Auxologico Italiano of Italy and from Institut Electricité-Santé of France.

  • Competing interests None.

  • Ethics approval Ethics committee approval obtained.

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