The Euro-Phospholipid project: epidemiology of the antiphospholipid syndrome in Europe

Abstract

The Euro-Phospholipid project started in 1999 with a multicentre, consecutive and prospective design. A total cohort of 1000 patients with antiphospholipid syndrome (APS), derived from 13 countries (Belgium, Bulgaria, Denmark, France, Germany, Greece, Hungary, Israel, Italy, the Netherlands, Portugal, Spain and United Kingdom), has been followed since then. This project allowed the identification of the prevalence and characteristics of the main clinical and immunological manifestations at the onset and during the evolution of APS and demonstrated that it is possible to recognize more homogeneous subsets of clinical significance. Patients with APS associated with systemic lupus erythematosus (SLE) had more episodes of arthritis, livedo reticularis and more frequently exhibited thrombocytopenia and leucopenia. Female patients had more episodes of arthritis and livedo reticularis - both connected with the higher prevalence of migraine and SLE-related APS in women, while male patients had more myocardial infarction, epilepsy and lower limb arterial thrombosis. Childhood onset patients presented more episodes of chorea and jugular vein thrombosis, whereas older onset patients were more frequently male and had more strokes and angina pectoris, but less frequently livedo reticularis.

Reactivity against the Five Domains of β2 Glycoprotein I: A Focus on Systemic Lupus Erythematosus

“…In particular, the aPL positivity rate among subjects with SLE ranges from 12% to 44% for aCL, from 15% to 34% for LA, and from 10% to 19% for anti-β 2 GPI antibodies [4]. In the Europhospholipid series, patients with full-blown SLE represented the 37% of the total cohort, while 4% of the included subjects presented a lupus-like disease [5]. Longitudinal studies show that APS may develop in 50 to 70% of aPL positive SLE patients after 20 years of follow-up.…”

Resolutive pulmonary endarterectomy in a non-compliant patient with systemic lupus erythematosus and antiphospholipid syndrome

“…Fifty per cent of APS patients have an associated connective tissue disease, mainly systemic lupus erythematosus (SLE) [1]. Pulmonary complications, especially pulmonary arterial hypertension (PAH), may aggravate both of these conditions: 0.5-14% in SLE [2] and 0.5% in APS [3].…”

European Working Party on Systemic Lupus Erythematosus and European Forum on Antiphospholipid Antibodies: two networks promoting European research on autoimmunity

“…This was the first epidemiological study performed all over the world on a large cohort of patients with APS and it provided information about the classical and nonclassical clinical aspects, the relationship between SLE and APS, etc., as detailed in the section of the journal devoted to this project. 18 The most original part of this work has been recently published and deals with the 5 years followup of the 1000 patients. 19 Information about morbidity and mortality of a population with a mean age of 42 years underline the impact of this disease on patient outcome.…”