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Primary Antiphospholipid Syndrome

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Front. Immunol. | doi: 10.3389/fimmu.2019.00941

Cardiac Manifestations of Antiphospholipid Syndrome with Focus on its Primary Form

Tamara Kolitz1, Shachaf Shiber2, 3, Itzhak Sharabi1, 3, Asher Winder1, 3 and  Gisele Zandman-Goddard1, 3*
  • 1Wolfson Medical Center, Israel
  • 2Rabin Medical Center, Israel
  • 3Sackler School of Medicine, Sackler Faculty of Medicine, Tel Aviv University, Israel

Antiphospholipid syndrome (APS) is a multisystem autoimmune disease most commonly associated with recurrent arterial and venous thromboembolism and recurrent fetal loss. Other possible antiphospholipid antibody (aPL)-related clinical manifestations include cardiac involvement. The heart is involved through direct (autoimmune mediated) and indirect (thrombosis) mechanisms. There is an increased cardiovascular mortality in APS; however, the cardiovascular risk is lower in patients with primary APS (PAPS) compared with lupus-related APS. Antiphospholipid syndrome (APS) is a multisystem autoimmune disease most commonly associated with recurrent arterial and venous thromboembolism and recurrent fetal loss. Other possible antiphospholipid antibody (aPL)-related clinical manifestations include cardiac involvement. The heart can be involved through immune mediated and /or thrombotic mechanisms. Mortality due to cardiovascular problems is elevated in APS. However, the cardiovascular risk in patients with primary APS (PAPS) compared with lupus-related APS is yet to be established. Cardiac symptoms of APS include valve abnormalities (thickening and vegetations), coronary artery disease (CAD), myocardial dysfunction, pulmonary hypertension and intracardiac thrombi. Heart valve lesions are the most common cardiac manifestation, observed in approximately one third of PAPS patients and usually do not cause hemodynamic significance. Deposits of immunoglobulins including anticardiolipin (aCL), and of complement components, are commonly observed in affected heart valves from these patients. This suggests that an inflammatory process is initiated by aPL deposition, eventually resulting in the formation of valvular lesion. aPL may have a direct role in the atherosclerotic process via induction of endothelial activation. Multiple traditional and autoimmune-inflammatory risk factors are involved in triggering an expedited atherosclerotic arterial disease evident in APS. It is imperative to increase the efforts in early diagnosis, control of risk factors and close follow-up, in the attempt to minimize cardiovascular risk in APS. Clinicians should bear in mind that a multidisciplinary therapeutic approach is of paramount importance in these patients. This article reviews the cardiac detriments of APS, including treatment recommendations for each cardiac complication.

Keywords: primary APS, Cardiac manifestations, Heart involvement, Valvular disease, Endocarditis

Received: 16 Nov 2018; Accepted: 11 Apr 2019.

Edited by:

Ricard Cervera, Departamento de Enfermedades Autoinmunes, Hospital Clínic de Barcelona, Spain

Reviewed by:

Anisur Rahman, University College London, United Kingdom
Maria G. Tektonidou, School of Health Sciences, National and Kapodistrian University of Athens, Greece  

Copyright: © 2019 Kolitz, Shiber, Sharabi, Winder and Zandman-Goddard. 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. Gisele Zandman-Goddard, Sackler School of Medicine, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel, goddard@wmc.gov.il