Damage measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in antiphospholipid antibody-positive patients included in the APS ACTION registry

Abstract

Objectives Our primary objective was to quantify damage burden measured by Damage Index for Antiphospholipid Syndrome (DIAPS) in antiphospholipid antibody (aPL)-positive patients with or without a history of thrombosis in an international cohort. Secondly, we aimed to identify clinical and laboratory characteristics associated with damage in aPL-positive patients. Methods In this cross-sectional study, we analyzed the baseline damage in aPL-positive patients with or without APS classification. We excluded patients with other autoimmune diseases. We analyzed the demographic, clinical, and laboratory characteristics based on two subgroups: (1) thrombotic APS patients with high versus low damage; and (2) non-thrombotic aPL-positive patients with versus without damage. Results Of the 826 aPL-positive patients included in the registry as of April 2020, 576 with no other systemic autoimmune diseases were included in the analysis (412 thrombotic and 164 non-thrombotic). In the thrombotic group, hyperlipidemia (OR 1.82, 95%CI 1.05–3.15, adjusted p= 0.032), obesity (OR 2.14, 95%CI 1.23–3.71, adjusted p= 0.007), aβ2GPI high titers (OR 2.33, 95%CI 1.36–4.02, adjusted p= 0.002), and corticosteroids use (ever) (OR 3.73, 95%IC 1.80–7.75, adjusted p< 0.001) were independently associated with high damage at baseline. In the non-thrombotic group, hypertension (OR 4.55, 95%CI 1.82–11.35, adjusted p= 0.001) and hyperlipidemia (OR 4.32, 95%CI 1.37–13.65, adjusted p= 0.013) were independent predictors of damage at baseline; conversely, single aPL positivity was inversely correlated with damage (OR 0.24; 95%CI 0.075–0,77, adjusted p= 0.016). Conclusions DIAPS indicates substantial damage in aPL-positive patients in APS ACTION cohort. Selected traditional cardiovascular risk factors, steroids use and specific aPL profiles may help to identify patients more prone to present with a higher damage burden.

Predictors of damage accrual and its impact on health-related quality of life of thrombotic antiphospholipid syndrome: Independent validation of the damage index for antiphospholipid syndrome (DIAPS)

“…20 In our study, the mean cumulative DIAPS was 1.48 (1.99) (median 0, IQR 0–2; Table 1) which is line with previously reported findings. 5,10,18–20 However, it is worth noting that only approximately 48% of our patients presented with damage. This heterogeneity may stem from variations between populations, local patient referencing practices or differences in study methodologies.…”

“…Consistent with previous studies, 8,10,21 our findings indicate that the neuropsychiatric domain is the most affected, followed by the peripheral vascular domain. Contrary reports were found by Torricelli et al 18 and in the APS ACTION cohort, 20 who reported the peripheral vascular domain as the most affected. Overall, these results are consistent with the fact that damage is expected from the main thrombotic manifestations (i.e.…”

“…19 Recently, DIAPS was evaluated in a multi-ethnic international cohort comprising 826 aPL-positive patients from the APS Alliance for Clinical Trials and International Networking (APS ACTION) registry. 20 Among the 412 pAPS group, 348 (84.5%) presented with damage and 110 (26.7%) with high damage (DIAPS≥3) at the baseline evaluation; the mean DIAPS value was 1.94 (1.46) and the median DIAPS was 2 (IQR 1–3, min 0, max 9). 20 In our study, the mean cumulative DIAPS was 1.48 (1.99) (median 0, IQR 0–2; Table 1) which is line with previously reported findings.…”

“…20 Among the 412 pAPS group, 348 (84.5%) presented with damage and 110 (26.7%) with high damage (DIAPS≥3) at the baseline evaluation; the mean DIAPS value was 1.94 (1.46) and the median DIAPS was 2 (IQR 1–3, min 0, max 9). 20 In our study, the mean cumulative DIAPS was 1.48 (1.99) (median 0, IQR 0–2; Table 1) which is line with previously reported findings. 5,10,18–20 However, it is worth noting that only approximately 48% of our patients presented with damage.…”

“…In the APS ACTION cohort, the presence of classical cardiovascular (CVD) risk factors, namely, male sex, older age, hypertension, hyperlipidaemia and obesity, were associated with higher damage. 20 This is reinforced in a cluster analysis conducted by Uludağ et al, where the authors identified a group ( n = 74) comprising older patients with CVD risk factors and a higher incidence of arterial events who exhibited a mean DIAPS of 2.24 ± 1.44, ranking second among the four clusters identified in terms of damage. 22 In our study, however, we did not observe any significant association between medical disorders (such as hypertension, obesity, diabetes and cardiovascular disease), as well as sex and age, with damage accrual (Table 2).…”