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Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients
  1. R Cervera1,
  2. R Serrano1,
  3. G J Pons-Estel1,
  4. L Ceberio-Hualde2,
  5. Y Shoenfeld3,
  6. E de Ramón4,
  7. V Buonaiuto4,
  8. S Jacobsen5,
  9. M M Zeher6,
  10. T Tarr6,
  11. A Tincani7,
  12. M Taglietti7,
  13. G Theodossiades8,
  14. E Nomikou8,
  15. M Galeazzi9,
  16. F Bellisai9,
  17. P L Meroni10,
  18. R H W M Derksen11,
  19. P G D de Groot12,
  20. M Baleva13,
  21. M Mosca14,
  22. S Bombardieri14,
  23. F Houssiau15,
  24. J-C Gris16,
  25. I Quéré16,
  26. E Hachulla17,
  27. C Vasconcelos18,
  28. A Fernández-Nebro19,
  29. M Haro19,
  30. Z Amoura20,
  31. M Miyara20,
  32. M Tektonidou21,
  33. G Espinosa1,
  34. M L Bertolaccini2,
  35. M A Khamashta2,
  36. on behalf of the Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies)
  1. 1Departament of Autoimmune Diseases, Hospital Clínic, Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Barcelona, Catalonia, Spain
  2. 2Lupus Unit, Rayne Institute, St Thomas’ Hospital, London, UK
  3. 3Zabludowicz Centre for Autoimmune Diseases, Chaim-Sheba Medical Centre, Sackler Faculty of Medicine Tel Aviv University, Tel-Hashomer, Israel
  4. 4Unidad de Enfermedades Autoinmunes Sistémicas, Servicio de Medicina Interna, Hospital Carlos Haya, Málaga, Spain
  5. 5Departament of Infectious Diseases and Rheumatology, Copenhagen University Hospital at Rigshospitalet, Copenhagen, Denmark
  6. 63rd Departament of Medicine, University of Debrecen, Medical and Health Science Centre, Debrecen, Hungary
  7. 7Servizio di Inmunologia Clinica e Allergologia, Spedali Civili, Azienda Ospidaliera, Brescia, Italy
  8. 8Transfusion and Haemophilia Centre, Hippocration Hospital, Athens, Greece
  9. 9Unità Operativa Complessa di Reumatologia, Azienda Ospedaliera Universitaria Senese, Siena, Italy
  10. 10Allergy and Clinical Immunology Unit, Dipartimento di Medicina Interna, IRCCS Istituto Auxologico, Università di Milano, Milan, Italy
  11. 11Department of Rheumatology and Clinical Immunology, University Medical Centre, Utrecht, The Netherlands
  12. 12Department of Haematology, Laboratory of Thrombosis and Haemostasis, University Medical Centre, Utrecht, The Netherlands
  13. 13Laboratory of Clinical Immunology, Clinical Centre of Allergology, Medical University, Sofia, Bulgaria
  14. 14Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy
  15. 15Service de Rhumatologie, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
  16. 16Laboratoire dHématologie, CHU, Nîmes, France
  17. 17Service de Médecine Interne, Hôpital Claude Huriez, Université Lille Nord-de-France, Lille, France
  18. 18Unidade de Imunologia Clínica, Hospital Santo António, Centro Hospitalar do Porto and UMIB, ICBAS, Universidade do Porto, Portugal
  19. 19Department of Rheumatology, Hospital Regional Universitario de Málaga, University of Málaga, IBIMA, Málaga, Spain
  20. 20Department of Internal Medicine, French National Reference Center for Lupus and Antiphospholipid Syndrome, Hôpital Pitié-Salpetrière, Paris, France
  21. 21First Department of Internal Medicine, University of Athens School of Medicine, Athens, Greece
  1. Correspondence to Dr Ricard Cervera, Servei de Malalties Autoimmunes, Hospital Clinic, Villarroel 170, Barcelona, Catalonia 08038, Spain; rcervera{at}clinic.cat

Abstract

Objectives To assess the prevalence of the main causes of morbi-mortality in the antiphospholipid syndrome (APS) during a 10-year-follow-up period and to compare the frequency of early manifestations with those that appeared later.

Methods In 1999, we started an observational study of 1000 APS patients from 13 European countries. All had medical histories documented when entered into the study and were followed prospectively during the ensuing 10 years.

Results 53.1% of the patients had primary APS, 36.2% had APS associated with systemic lupus erythematosus and 10.7% APS associated with other diseases. Thrombotic events appeared in 166 (16.6%) patients during the first 5-year period and in 115 (14.4%) during the second 5-year period. The most common events were strokes, transient ischaemic attacks, deep vein thromboses and pulmonary embolism. 127 (15.5%) women became pregnant (188 pregnancies) and 72.9% of pregnancies succeeded in having one or more live births. The most common obstetric complication was early pregnancy loss (16.5% of the pregnancies). Intrauterine growth restriction (26.3% of the total live births) and prematurity (48.2%) were the most frequent fetal morbidities. 93 (9.3%) patients died and the most frequent causes of death were severe thrombosis (36.5%) and infections (26.9%). Nine (0.9%) cases of catastrophic APS occurred and 5 (55.6%) of them died. The survival probability at 10 years was 90.7%.

Conclusions Patients with APS still develop significant morbidity and mortality despite current treatment. It is imperative to increase the efforts in determining optimal prognostic markers and therapeutic measures to prevent these complications.

  • Anticardiolipin Antibodies
  • Antiphospholipid Antibodies
  • Antiphospholipid Syndrome
  • Autoimmune Diseases
  • Systemic Lupus Erythematosus

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