Abstract
Cognitive impairment is frequently reported among anti-phospholipid syndrome (APS) patients as well as anti-phospholipid antibody (aPL) carriers, but it is less studied than other manifestations of this condition. Moreover, the exact prevalence of cognitive impairment in these patients has not been accurately determined, mainly due to inconsistency in the tools used to identify impairment, small sample sizes, and variability in the anti-phospholipid antibodies measured and positivity cutoffs. The notion of a direct pathogenic effect is supported by the observation that the higher the number of aPLs present and the higher the load of the specific antibody, the greater the risk of cognitive impairment. There is some evidence to suggest that besides the thrombotic process, inflammation-related pathways play a role in the pathogenesis of cognitive impairment in APS. The cornerstone treatments of APS are anti-coagulant and anti-thrombotic medications. These treatments have shown some favorable effects in reversing cognitive impairment, but solid evidence for the efficacy and safety of these treatments in the context of cognitive impairment is still lacking. In this article, we review the current knowledge regarding the epidemiology, pathophysiology, clinical associations, and treatment of cognitive impairment associated with APS and aPL positivity.
Highlights
Anti-phospholipid syndrome (APS) is an acquired systemic disorder associated with the presence of anti-phospholipid antibodies
Several case reports showed that dementia and other neurologic features of anti-phospholipid syndrome (APS) may be reversible through the initiation of immunosuppression, emphasizing the possibility of inflammatory pathways acting as driving mechanisms for cognitive impairment [46]
These findings suggest that autoimmune mechanisms may underlie, at least partially, the cognitive impairment observed in patients with aPLs
Summary
Anti-phospholipid syndrome (APS) is an acquired systemic disorder associated with the presence of anti-phospholipid antibodies (aPLs). Most studies examining cognitive impairment in aPL carriers and in APS have included a small sample size and varied considerably in terms of cognitive impairment detection methods, the particular aspects of cognition evaluated, and the specific antibody type (aCL, LA or aβ2GPI) and the laboratory cutoffs used to define positivity [5]. This complexity in the interpretation of the results is further increased by the following (Table 1): firstly, aPL can be found in the general population with a prevalence of 1–5% [5]. Several tests have been used for assessing cognitive impairment and dementia, but the Mini-Mental State Examination (MMSE) and the Montreal Cognitive Assessment (MoCA) are most commonly used, with a sensitivity of 75–92% and a specificity of 81–91%, respectively [8]
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