Disability and cognitive impairment are interdependent in primary antiphospholipid syndrome

Abstract

Background Cognitive impairment (CI) occurs at a high frequency in primary antiphospholipid syndrome (PAPS). Its psychosocial-related factors are of interest. Objective We aimed to determine disability and perceived stress and their correlation with CI in PAPS. Methods First study phase: a longitudinal study including patients with PAPS and paired controls for cardiovascular risk factors, age, and sex, determining CI with Montreal Cognitive Assessment (MoCA) and then repeating the measurement 1 year later. Second study phase: a cross-sectional analytical study by quantification of disability with the World Health Organization Disability Assessment Schedule (WHODAS 2.0) and perceived stress with the Perceived Stress Scale (PSS-14). Descriptive statistics and Spearman correlation coefficient were used. Results Sixty-three patients with PAPS and 60 controls were studied. In PAPS, age (range, 48.0 ± 13.5 years), thrombotic artery events (TAE) (44.4%), and stroke/TIA (42.8%) were found. Disability was documented in the majority of WHODAS 2.0 domains and the total score for this was higher in participation and mobility, the stress level was normal, and 65.1% had CI. PAPS exhibited greater deterioration in the WHODAS 2.0 total score ( p .017) and the MoCA test ( p < .0001). Personal domains and the total WHODAS 2.0 score correlated inversely with MoCA. Life activities ( rho = –0.419) and self-care ( rho = –0.407) were those that correlated to the greatest degree. Stroke conferred risk for CI. Conclusions Disability in PAPS and CI are interdependent. New treatment options and neurocognitive stimulation strategies are necessary to maintain functionality and prevent further cognitive dysfunction in PAPS patients.

A one-point increase in the Damage Index for Antiphospholipid Syndrome (DIAPS) predicts mortality in thrombotic antiphospholipid syndrome

“…Most patients with thrombotic APS have at least one point scored in DIAPS in a long-term basis. In our population, nearly 73% of patients developed damage with a median last DIAPS of 1 (IQR 2 -0) at the end of follow-up, but prevalence higher than 90% has been reported (10,12). In contrast, only 29% of patients developed damage using SDI, with a median SDI score of 2 (IQR 3 -1) after a median of 7.5 years of follow-up (5).…”

“…For instance, peripheral vascular domain involvement is by far more frequent among APS (more than 60%) (10,12) than in SLE patients (around 10%) (17), in particular, vascular venous insufficiency which is responsible for nearly 42% of those events (10). Although it highly impairs quality of life (10,18), it is arguably very unlikely to influence survival.…”

“…For instance, peripheral vascular domain involvement is by far more frequent among APS (more than 60%) (10,12) than in SLE patients (around 10%) (17), in particular, vascular venous insufficiency which is responsible for nearly 42% of those events (10). Although it highly impairs quality of life (10,18), it is arguably very unlikely to influence survival. On the other hand, chronic kidney disease which affects almost 50% of SLE patients (19), and also some treatment-related damage like diabetes, affecting around 5% of SLE patients (17,20), are known risk factor for mortality in general population (21,22).…”

“…In 2015, Amigo and colleagues (9) published a new scoring instrument, the damage index for APS (DIAPS), specifically designed to overcome some of the SDI limitations with regard of assessing the full damage picture of thrombotic APS. The DIAPS is a 38-item score that has demonstrated content, criterion and construct validity as well as a significant positive high correlation with impaired health-related quality of life in Latin Americans (9,10).…”

“…No similar fact is known for thrombotic APS. Only a few studies have investigated factors associated with damage development, its quantification and kinetics in APS (4,5,9,10,12). Moreover, there has been no correlation between the damage acquisition measured by the DIAPS with mortality after its initial development and validation.…”