Abstract
Antiphospholipid syndrome (APS) is characterized by arterial and venous thrombosis, pregnancy morbidity and fetal loss caused by pathogenic autoantibodies directed against phospholipids (PL) and PL-cofactors. Isolated neurological APS may represent a significant diagnostic challenge, as epidemiological, clinical and neuroimaging features may overlap with those of multiple sclerosis (MS). In an open view, MS could be considered as an organ-specific anti-lipid (phospholipid and glycosphingolipid associated proteins) disease, in which autoreactive B cells and CD8+ T cells play a dominant role in its pathophysiology. In MS, diverse autoantibodies against the lipid-protein cofactors of the myelin sheath have been described, whose pathophysiologic role has not been fully elucidated. We carried out a review to select clinical studies addressing the prevalence of antiphospholipid (aPL) autoantibodies in the so-called MS-like syndrome. The reported prevalence ranged between 2% and 88%, particularly aCL and aβ2GPI, with predominant IgM isotype and suggesting worse MS prognosis. Secondarily, an updated summary of current knowledge on the pathophysiological mechanisms and events responsible for these conditions is presented. We draw attention to the clinical relevance of diagnosing isolated neurological APS. Prompt and accurate diagnosis and antiaggregant and anticoagulant treatment of APS could be vital to prevent or at least reduce APS-related morbidity and mortality.
Highlights
Chiara D’Angelo 1,2,3, Oriol Franch 4, Lidia Fernández-Paredes 1,2, Celia Oreja-Guevara 5, María Núñez-Beltrán 1, Alejandra Comins-Boo 1,2, Marcella Reale 3 and Silvia Sánchez-Ramón 1,2*
multiple sclerosis (MS) could be considered as an organ-specific anti-lipid disease, in which autoreactive B cells and CD8+ T cells play a dominant role in its pathophysiology
We summarize the events taking place in both innate and adaptive responses concerning Antiphospholipid syndrome (APS)
Summary
APS is characterized by the presence of autoantibodies, but as previously mentioned, aPL antibodies is a necessary but not sufficient condition for the onset of the disease. APL antibodies directly promote up-regulation of the TF synthesis in monocytes (Sorice et al, 2007), neutrophils (Ritis et al, 2006) and endothelial cells (Kornberg et al, 2000) This procoagulant condition, not present under normal circumstances, plays an important role in contributing to the FIGURE 2 | Schematic representation of the autoreactive response in APS in which the thrombus formation is mediated by the interaction of antiphospholipid (aPL) antibodies with the anticoagulant annexinV (AnV) on the surface of the endothelial cells, which are destroyed, accelerating coagulation cascade. Absence or reduction of NKT cells or of CD1d-expression on B cells have been related with an increased autoreactive B cells activation and higher aPL release, which raised the hypothesis that aPL antibodies could result from imbalanced NKT population These data point to NKT cells as an intriguing therapeutic strategy to establish tolerance, a key element for ameliorating autoimmune diseases (Wermeling et al, 2010)
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