Abstract

Background:Antiphospholipid syndrome (APS) is a rare autoimmune disorder that mainly affects young individuals. Due to the potentially devasting effects of some of its clinical manifestations, such as ischemic stroke and thrombotic microangiopathy, APS can have a deep negative impact on the quality of life (QoL) of the affected patients due to damage accrual (2).To capture the full consequences of the disease, including both thrombotic and extra-criteria APS specific features, the antiphospholipid syndrome (APS) damage index (DIAPS), a 37-item score, was conceived.Objectives:The aim of this study was to validate the DIAPS in a cohort of antiphospholipid antibodies (aPL) positive subjects, comparing it to the risk stratification score for the development of clinical manifestations of APS (Global APS Score -GAPSS) and QoL questionnaires filled out by APS patients.Methods:This retrospective study included a total of 84 consecutive persistent aPL positive patients who attended the San Giovanni Bosco Hospital (Turin, Italy). Patients were then divided based on their diagnosis in 3 groups: primary APS (PAPS), secondary APS (SAPS) and aPL positive patients without clinical manifestations of APS, according to Miyakis criteria (1). Demographic, clinical and laboratory characteristics, were retrieved from electronic medical records and are summarized in Table 1. DIAPS was calculated as previously reported by adding together all points corresponding to the clinical manifestations of the patients (3).Results:A total of consecutive 84 patients were enrolled in the study: 39 primary APS patients (PAPS), 28 secondary APS patients (SAPS) and 17 aPL positive patients without APS-related clinical manifestations. APS patients had significantly higher levels of DIAPS when compared to aPL asymptomatic patients (mean DIAPS 2.6 ±1.8 vs. 1.5 ±1.9, respectively; p<0.05). Moreover, SAPS patients had significantly higher levels of DIAPS when compared to PAPS patients (mean DIAPS 3.1 ±1.9 vs. 2.2 ±1.7, respectively; p<0.05). When comparing GAPSS and DIAPS levels in all the patients, we found a significantly positive correlation between these two scores (mean GAPSS 11.9 ±5.2 and mean DIAPS 2.4 ±1.9; Pearson 0.241; p<0.05). Finally, when applying the SF-12 score to our cohort, the mean physical component score and mental component score were lower than the average population (39.3 ±11.3 and 42.3 ±8, respectively) and we observed a negative correlation trend between DIAPS and both PCS and MCS (Pearson -0.133 and -0.183).Conclusion:The DIAPS might be a valid score system for the assessment of APS-related damage and QoL.

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