Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients
- R Cervera1,
- M A Khamashta2,
- Y Shoenfeld3,
- M T Camps4,
- S Jacobsen5,
- E Kiss6,
- M M Zeher6,
- A Tincani7,
- I Kontopoulou-Griva8,
- M Galeazzi9,
- F Bellisai9,
- P L Meroni10,
- R H W M Derksen11,
- P G de Groot12,
- E Gromnica-Ihle13,
- M Baleva14,
- M Mosca15,
- S Bombardieri15,
- F Houssiau16,
- J-C Gris17,
- I Quéré17,
- E Hachulla18,
- C Vasconcelos19,
- B Roch20,
- A Fernández-Nebro21,
- J-C Piette22,
- G Espinosa1,
- S Bucciarelli1,
- C N Pisoni2,
- M L Bertolaccini2,
- M-C Boffa22,
- G R V Hughes2
- 1Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain
- 2Lupus Unit, Rayne Institute, St Thomas’ Hospital, London, UK
- 3Research Centre for Autoimmune Diseases, Chaim-Sheba Medical Centre, Tel-Hashomer, Israel
- 4Unidad de Enfermedades Autoinmunitarias Sistémicas, Servicio de Medicina Interna, Hospital Regional “Carlos Haya”, Málaga, Spain
- 5Department of Rheumatology, Copenhagen University Hospital at Rigshospitalet, Copenhagen, Denmark
- 63rd Department of Medicine, University of Debrecen, Medical and Health Science Centre, Debrecen, Hungary
- 7Servizio di Immunologia Clinica e Allergologia, Spedali Civili, Azienda Ospedaliera, Brescia, Italy
- 8Transfusion and Haemophilia Centre, Hippocration Hospital, Athens, Greece
- 9Istituto di Reumatologia, Policlinico “Le Scotte”, Siena, Italy
- 10Allergy and Clinical Immunology Unit, Dipartimento di Medicina Interna, IRCCS Istituto Auxologico, Università di Milano, Milan, Italy
- 11Department of Rheumatology and Clinical Immunology, University Medical Centre, Utrecht, The Netherlands
- 12Department of Haematology, Laboratory of Thrombosis and Haemostasis, University Medical Centre, Utrecht, The Netherlands
- 13Rheumaklinik Berlin-Buch, Immanuel-Krankenhaus GmbH, Berlin, Germany
- 14Laboratory of Clinical Immunology, Clinical Centre of Allergology, Medical University, Sofia, Bulgaria
- 15Dipartimento di Medicina Interna, Università di Pisa, Pisa, Italy
- 16Service de Rhumatologie, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium
- 17Laboratoire dHématologie, CHU, Nìmes, France
- 18Service de Médecine Interne, Hôpital Claude Huriez, Lille, France
- 19Departamento de Medicina Interna, Hospital Geral San António, Porto, Portugal
- 20Rheumatologische Ambulanz, Medizinische Klinik III und Medizinische Poliklinik, Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
- 21Sección de Reumatología, Hospital Clínico Universitario, Málaga, Spain
- 22Department of Internal Medicine, Hôpital Pitié-Salpêtrière, Paris, France
- Correspondence to Dr R Cervera, Servei de Malalties Autoimmunes, Hospital Clínic, Villarroel 170, 08036-Barcelona, Catalonia, Spain;
- Accepted 5 September 2008
- Published Online First 18 September 2008
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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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.