Abstract
Antiphospholipid syndrome (APS) is an autoimmune antibody-mediated condition characterized by thrombotic events and/or pregnancy morbidity in association with persistent positivity to antiphospholipid antibodies (aPL). The nervous system is frequently affected, as intracranial vessels are the most frequent site of arterial pathology. Over the course of years, many other neurological conditions not included in the diagnostic criteria, have been associated with APS. The pathogenic mechanisms behind the syndrome are complex and not fully elucidated. aPL enhance thrombosis, interfering with different pathways. Nevertheless, ischemic injury is not always sufficient to explain clinical features of the syndrome and immune-mediated damage has been advocated. This may be particularly relevant in the context of neurological complications. The reason why only a subgroup of patients develop non-criteria nervous system disorders and what determines the clinical phenotype are questions that remain open. The double nature, thrombotic and immunologic, of APS is also reflected by therapeutic strategies. In this review we summarize known neurological manifestations of APS, revisiting pathogenesis and current treatment options.
Highlights
Antiphospholipid syndrome (APS) is an autoimmune antibody-mediated disorder defined by the occurrence of thrombosis and/or pregnancy morbidity in presence of persistent antiphospholipid antibodies [1]
In this paper we summarize current concepts regarding the pathogenesis of APS, reviewing neurological clinical features and related therapeutic implications
Some authors have suggested that aPL may alter the integrity of the blood-brain barrier (BBB) and facilitate the access of immune cells to the central nervous system (CNS) compartment [81, 82]
Summary
Antiphospholipid syndrome (APS) is an autoimmune antibody-mediated disorder defined by the occurrence of thrombosis and/or pregnancy morbidity in presence of persistent antiphospholipid antibodies (aPL) [1]. The diseases can occur alone (primary APS) or in the context of other autoimmune conditions, in particular systemic lupus erythematosus (SLE), Sjögren’s syndrome and rheumatoid arthritis (secondary APS). Pregnancy morbidity includes embryonic losses, fetal death, and premature birth. The diagnosis is made according to the updated international Sydney consensus criteria (Table 1) [1]. Patients with persistent aPL may present with clinical manifestations not included in the criteria (the so called “non-criteria” symptoms), among which thrombocytopenia, hemolytic anemia, cardiac valve disease, renal microangiopathy, livedo reticularis, and neurologic disturbances other than ischemic cerebrovascular accidents (CVA) [3]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
