Background Though the probability of survival at 10 years is 90.7% in the antiphospholipid syndrome (APS), eventual haemostatic of immunological predictors of mortality in primary APS (PAPS) have been poorly investigated.
Objectives To identify mortality predictors in patients with PAPS.
Methods Record review of all PAPS patients attending the Haemostasis Unit of the Cardarelli Hospital from 1988 to 2008. We identified 11 deceased patients (5M, 6F, age 55±9 yrs), 68 surviving thrombotic PAPS (21M, 47F, 47±14 yrs) and 29 persistent carriers of antiphospholipid antibodies (PCaPL) without thrombosis (7M, 22F,tage 50±16 yrs). The following had been tested at baseline and confirmed 6 weeks later: aPTT, DRVVT, KCT and plasma fibrinogen (FNG) by coagulation assays; IgG anticardiolipin (aCL) and von Willebrand factor (vWF) by Elisa; platelet (PLT) count on automatic analyser.
Results IgG aCL (GPL) was 328±344 in deceased PAPS, 105±146 in surviving PAPS and 75±167 in PCaPL (p<0.0001); aPTT ratio was 2.17±0.46 in deceased PAPS, 1.92±0.65 in surviving PAPS and 1.79±1.01 in PCaPL (p<0.02); DRVVT ratio was 2.30±0.80 in deceased PAPS, 1.65±0.43 in surviving PAPS and 1.33±0.32 in PCaPL (p<0.0001); plasma FNG (mg/dl) was 385±70 in deceased PAPS, 335±49 in surviving PAPS and 312±63 in PCaPL (p<0.02); PLTs (109/L) were 293±105 in deceased PAPS, 202±86 in surviving PAPS and 141±77 in PCaPL (p<0.0001). KCT ratio and vWF concentration did not differ. Sex, disease duration, age at first thrombotic event, number of thrombotic events, IgG aCL titre, aPTTr, DRVVTr, FNG, vWF and platelet numbers were entered as independent variables in a stepwise Cox regression model with mortality as the dependent variable: DRVVTr was the single most powerful predictor of mortality that was 20% at 17 years.
Conclusions DRVVTr is an independent predictor of mortality: efforts of future research should be aimed at measures to decrease antiphospholipid generation.
Cervera R, Serrano R, Pons-Estel GJ et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2014 Jan 24
Disclosure of Interest None declared