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Thromb Haemost 2016; 115(06): 1200-1208
DOI: 10.1160/TH15-10-0839
Stroke, Systemic or Venous Thromboembolism
Schattauer GmbH

Survival in primary antiphospholipid syndrome

A single-centre cohort study
Paul R. J. Ames
1   Multimedica SRL, Naples, Italy
2   Immune Response and Vascular Disease Unit, Nova University, Lisbon, Portugal
,
Mira Merashli
3   Muskuloskeletal Biomedical Research Unit, Leeds Teaching Hospitals NHS Trust, Leeds, UK
,
Irina Chis Ster
4   Institute of Infection and Immunity, St George’s University of London, London, UK
,
Giovanna D’Andrea
5   Genetica Medica, Universita’ di Foggia, Foggia, Italy
,
Luigi Iannaccone
1   Multimedica SRL, Naples, Italy
,
Vincenzo Marottoli
1   Multimedica SRL, Naples, Italy
,
Maurizio Margaglione
5   Genetica Medica, Universita’ di Foggia, Foggia, Italy
,
Vincenzo Brancaccio
6   Ex Coagulation Unit, Antonio Cardarelli Hospital, Naples, Italy
› Author Affiliations Financial support: This study was supported by www.FondazioneAPS.it, an Italian registered charity.
Further Information

Publication History

Received: 30 October 2015

Accepted after major revision: 22 January 2016

Publication Date:
27 November 2017 (online)

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Summary

The vascular mortality of antiphospholipid syndrome (APS) ranges from 1.4 % to 5.5 %, but its predictors are poorly known. It was the study objective to evaluate the impact of baseline lupus anticoagulant assays, IgG anticardiolipin (aCL), plasma fibrinogen (FNG) and von Willebrand factor (VWF), platelets (PLT) and of genetic polymorphisms of methylenetetrahydrofolate reductase C677T, of prothrombin G20210A and of paraoxonase-1 Q192R on survival in primary APS (PAPS). Cohort study on 77 thrombotic PAPS and 33 asymptomatic carriers of aPL (PCaPL) seen from 1989 to 2015 and persistently positive for aPL as per annual review. At baseline all participants were tested twice for the ratios of kaolin clotting time (KCTr), activated partial thromboplastin time (aPTTr), dilute Russell viper venom time (DRVVTr), IgG aCL, FNG, VWF and once for PLT. All thrombotic PAPS were on warfarin with regular INR monitoring. During follow-up 11 PAPS deceased (D-PAPS) of recurrent thrombosis mostly arterial, despite adequate anticoagulation yielding an overall vascular mortality of 10 %. D-PAPS had the strongest baseline aPTTr and DRVVTr and the highest mean baseline IgG aCL, FNG, VWF and PLT. Cox proportional hazards model identified baseline DRVVTr and FNG as main predictors of mortality with adjusted hazard ratios of 5.75 (95 % confidence interval [CI]: 1.5, 22.4) and of 1.03 (95 %CI: 1.01, 1.04), respectively. In conclusion, plasma DRVVTr and FNG are strongly associated with the risk of vascular death in PAPS; while FNG lowering agents exist further research should be directed at therapeutic strategies able to dampen aPL production.

Keywords

Antiphospholipid syndrome - DRVVT - fibrinogen - mortality
 

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