Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients

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).

[1]  M. Petri,et al.  Aspirin for primary thrombosis prevention in the antiphospholipid syndrome: a randomized, double-blind, placebo-controlled trial in asymptomatic antiphospholipid antibody-positive individuals. , 2007, Arthritis and rheumatism.

[2]  L. Leibovici,et al.  Clusters of disease manifestations in patients with antiphospholipid syndrome demonstrated by factor analysis , 2007, Lupus.

[3]  Y. Shoenfeld,et al.  Analysis of risk factors for the development of thrombotic complications in antiphospholipid antibody positive lupus patients , 2007, Lupus.

[4]  G. Ruiz-Irastorza,et al.  Effect of antimalarials on thrombosis and survival in patients with systemic lupus erythematosus , 2006, Lupus.

[5]  G. Espinosa,et al.  Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. , 2006, Arthritis and rheumatism.

[6]  S. Iliceto,et al.  The Diagnosis of the Antiphospholipid Syndrome , 2006, Pathophysiology of Haemostasis and Thrombosis.

[7]  Y. Shoenfeld,et al.  International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS) , 2006, Journal of thrombosis and haemostasis : JTH.

[8]  Y. Shoenfeld,et al.  Infectious origin of the antiphospholipid syndrome , 2005, Annals of the rheumatic diseases.

[9]  Y. Shoenfeld,et al.  Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines , 2003, Lupus.

[10]  G. Hughes,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. , 2002, Archives of internal medicine.

[11]  J. Piette,et al.  Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. , 2002, Arthritis and rheumatism.

[12]  M. Petri,et al.  Catastrophic Antiphospholipid Syndrome: Clues to the Pathogenesis from a Series of 80 Patients , 2001, Medicine.

[13]  M. Petri Management of thrombosis in antiphospholipid antibody syndrome. , 2001, Rheumatic diseases clinics of North America.

[14]  T. Koike,et al.  Anticardiolipin Antibody Assay: a Methodological Analysis for a better Consensus in Routine Determinations , 2001, Thrombosis and Haemostasis.

[15]  G. Espinosa,et al.  Clinical study and follow-up of 100 patients withthe antiphospholipid syndrome , 1999 .

[16]  W A Wilson,et al.  International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. , 1999, Arthritis and rheumatism.

[17]  G. Hughes,et al.  Morbidity and mortality in systemic lupus erythematosus during a 5-year period - A multicenter prospective study of 1,000 patients , 1999 .

[18]  G. Espinosa,et al.  Clinical study and follow-up of 100 patients with the antiphospholipid syndrome. , 1999, Seminars in arthritis and rheumatism.

[19]  I. Scharrer,et al.  Criteria for the Diagnosis of Lupus Anticoagulants: An Update , 1995, Thrombosis and Haemostasis.

[20]  P. D. de Groot,et al.  Patients with antiphospholipid antibodies and venous thrombosis should receive long term anticoagulant treatment. , 1993, Annals of the rheumatic diseases.

[21]  L. S. King,et al.  Signs and symptoms. , 1968, JAMA.