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...
Main Authors: | , , , , |
---|---|
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 |
_version_ | 1819232145894277120 |
---|---|
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. |
first_indexed | 2024-12-23T11:56:13Z |
format | Article |
id | doaj.art-e64796c333e649c590161aca85871eb7 |
institution | Directory Open Access Journal |
issn | 1664-3224 |
language | English |
last_indexed | 2024-12-23T11:56:13Z |
publishDate | 2019-05-01 |
publisher | Frontiers Media S.A. |
record_format | Article |
series | Frontiers in Immunology |
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 |
work_keys_str_mv | AT tamarakolitz cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT shachafshiber cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT shachafshiber cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT itzhaksharabi cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT itzhaksharabi cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT asherwinder cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT asherwinder cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT giselezandmangoddard cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform AT giselezandmangoddard cardiacmanifestationsofantiphospholipidsyndromewithfocusonitsprimaryform |