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Front. Neurol. | doi: 10.3389/fneur.2018.01001

Antiphospholipid syndrome and the neurologist: from pathogenesis to therapy

 Thomas Fleetwood1, Roberto Cantello1 and  CRISTOFORO COMI1*
  • 1Azienda Ospedaliero Universitaria Maggiore della Carita, Italy

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.

Keywords: APS, Antiphospholipid sydrome, APL - autoantibodies against phospholipids and phospholipid-binding proteins, neurological manifestations, Pathogenic mechanisms, therapy

Received: 31 Jul 2018; Accepted: 06 Nov 2018.

Edited by:

Tatiana Koudriavtseva, Istituto Nazionale del Cancro Regina Elena, Italy

Reviewed by:

Noriko Isobe, Kyushu University, Japan
Danieli C. Andrade, University of São Paulo, Brazil
Luca Prosperini, Azienda Ospedaliera San Camillo-Forlanini, Italy  

Copyright: © 2018 Fleetwood, Cantello and COMI. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

* Correspondence: Prof. CRISTOFORO COMI, Azienda Ospedaliero Universitaria Maggiore della Carita, Novara, Italy, cristoforo.comi@med.uniupo.it