Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form

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 invol...

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Main Authors: Tamara Kolitz, Shachaf Shiber, Itzhak Sharabi, Asher Winder, Gisele Zandman-Goddard
Format: Article
Language:English
Published: Frontiers Media S.A. 2019-05-01
Series:Frontiers in Immunology
Subjects:
Online Access:https://www.frontiersin.org/article/10.3389/fimmu.2019.00941/full
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author Tamara Kolitz
Shachaf Shiber
Shachaf Shiber
Itzhak Sharabi
Itzhak Sharabi
Asher Winder
Asher Winder
Gisele Zandman-Goddard
Gisele Zandman-Goddard
author_facet Tamara Kolitz
Shachaf Shiber
Shachaf Shiber
Itzhak Sharabi
Itzhak Sharabi
Asher Winder
Asher Winder
Gisele Zandman-Goddard
Gisele Zandman-Goddard
author_sort Tamara Kolitz
collection DOAJ
description 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.
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spelling doaj.art-e64796c333e649c590161aca85871eb72022-12-21T17:48:04ZengFrontiers Media S.A.Frontiers in Immunology1664-32242019-05-011010.3389/fimmu.2019.00941437113Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary FormTamara Kolitz0Shachaf Shiber1Shachaf Shiber2Itzhak Sharabi3Itzhak Sharabi4Asher Winder5Asher Winder6Gisele Zandman-Goddard7Gisele Zandman-Goddard8Department of Medicine C, Wolfson Medical Center, Holon, IsraelDepartment of Rheumatology, Rabin Medical Center, Petach Tikva, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelDepartment of Cardiology, Wolfson Medical Center, Holon, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelDepartment of Hematology, Wolfson Medical Center, Holon, IsraelDepartment of Medicine C, Wolfson Medical Center, Holon, IsraelSackler Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelAntiphospholipid 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.https://www.frontiersin.org/article/10.3389/fimmu.2019.00941/fullprimary antiphospholipid syndromesecondary antiphospholipid syndromeAPS antiphospholipid antibodiescardiac manifestationscardiovascular diseaseheart valve disease
spellingShingle Tamara Kolitz
Shachaf Shiber
Shachaf Shiber
Itzhak Sharabi
Itzhak Sharabi
Asher Winder
Asher Winder
Gisele Zandman-Goddard
Gisele Zandman-Goddard
Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form
Frontiers in Immunology
primary antiphospholipid syndrome
secondary antiphospholipid syndrome
APS antiphospholipid antibodies
cardiac manifestations
cardiovascular disease
heart valve disease
title Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form
title_full Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form
title_fullStr Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form
title_full_unstemmed Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form
title_short Cardiac Manifestations of Antiphospholipid Syndrome With Focus on Its Primary Form
title_sort cardiac manifestations of antiphospholipid syndrome with focus on its primary form
topic primary antiphospholipid syndrome
secondary antiphospholipid syndrome
APS antiphospholipid antibodies
cardiac manifestations
cardiovascular disease
heart valve disease
url https://www.frontiersin.org/article/10.3389/fimmu.2019.00941/full
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