Pengo V, Ruffatti A, Legnani C, Gresele P, Barcellona D, Erba N, Testa S, Marongiu F, Bison E, Denas G, Banzato A, Padayattil Jose S, Iliceto S
Clinical course of high risk patients diagnosed with Antiphospholipid Syndrome (APS). [JOURNAL ARTICLE]
J Thromb Haemost 2009 Oct 30.
Abstract
Background: The characteristics and the clinical course of antiphospholipid syndrome (APS) in high risk patients, positive for all three recommended tests exploring the presence of antiphospholipid (aPL) antibodies, have not been described.
Methods: This retrospective analysis of prospectively collected data, considered patients followed in Italian Thrombosis Centers, diagnosed as having definite APS and triple positivity for aPL [lupus anticoagulant (LA), anticardiolipin (aCL), and anti-beta2-glycoprotein I (beta2GPI) antibodies]. Laboratory data were confirmed in a central reference laboratory.
Results: One hundred-sixty patients were enrolled in this cohort study. The qualifying event at diagnosis was venous thromboembolism in 76 cases (47.5%), arterial thromboembolism in 69 cases (43.1%) and pregnancy morbidity in 11 cases (9.7%%). The remaining 4 patients (2.5%) suffered from catastrophic APS. The cumulative incidence of thromboembolic events in the follow up period was 12.2% (95% CI, 9.6 to 14.8) after 1 year, 26.1% (95% CI, 22.3 to 29.9) after 5 years and 44.2% (95% CI, 38.6 to 49.8) after 10 years; and was significantly higher in patients not taking oral anticoagulants as compared to those on treatment (HR=2.4 95%CI 1.3-4.1; p<0.003). Major bleeding associated with oral anticoagulant therapy was low (0.8% patient /years). Ten patients died (7 were cardiovascular deaths).
Conclusions: Patients with APS and triple positivity for aPL are at high risk of developing future thromboembolic events. The recurrences remain frequent despite the use of oral anticoagulants, which significantly reduces the risk of thromboembolism.
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