Predictors of Mortality in Antiphospholipid Antibody-Positive Patients: Prospective Results From Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking Clinical Database and Repository.

Abstract

Objective: The objective was to determine the mortality rate as well as the causes and predictors of death in antiphospholipid antibody (aPL)-positive patients with and without antiphospholipid syndrome (APS) classification.

Methods: The inclusion criterion for the multicenter international Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) registry is positive aPLs according to the Revised Sapporo Classification Criteria tested within one year before enrollment. Patients are observed every 12 ± 3 months with clinical data and blood collection. For this prospective analysis, we first analyzed the causes of death for patients reported as "deceased." Secondly, we analyzed risk factors for death using the adjusted Cox proportional hazards model and calculated survival probability using the Kaplan-Meier model based on different age groups.

Results: Of 967 patients, 43 (5%) were deceased after a median follow-up of 5.3 years. Based on the univariate analysis, deceased patients, compared to living patients, were more likely to be older and have a history of arterial thrombosis, catastrophic APS, concomitant systemic autoimmune diseases (SAIDs), and baseline cardiovascular disease (CVD) risk factors. Based on the Cox proportional hazards model adjusted for age and for each of the strongest predictors of death, arterial thrombosis (hazard ratio [HR] 2.94, 95% confidence interval [CI] 1.50-5.76), concomitant SAIDs (HR 2.97, 95% 1.56-5.63), and baseline any CVD risk factor (HR 2.43, 95% CI 1.05-5.71) were significantly associated with mortality.

Conclusion: In our cohort of persistently aPL-positive patients, the mortality rate was 5% after a median follow-up of five years and was highest for patients >60 years old at registry entry. History of arterial thrombosis, concomitant SAIDs, and baseline any CVD risk factor independently predicted future death.