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Autoimmun Rev. 2021 Nov 02;102990. doi: 10.1016/j.autrev.2021.102990. Epub 2021 Nov 02.

Myocardial involvement in anti-phospholipid syndrome: Beyond acute myocardial infarction.

Autoimmunity reviews

Lavinia Agra Coletto, Maria Gerosa, Mariaconsuelo Valentini, Rolando Cimaz, Roberto Caporali, Pier Luigi Meroni, Cecilia Beatrice Chighizola

Affiliations

  1. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy; Division of Clinical Rheumatology, ASST G. Pini - CTO, Milan, Italy.
  2. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy; Division of Clinical Rheumatology, ASST G. Pini - CTO, Milan, Italy. Electronic address: [email protected].
  3. Cardiology Unit, San Luca Hospital, Istituto Auxologico Italiano, IRCCS, Milan, Italy.
  4. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy; Pediatric Rheumatology Unit, ASST G. Pini - CTO, Milan, Italy.
  5. Experimental Laboratory of Immunological and Rheumatologic Researches, Istituto Auxologico Italiano, IRCCS, Cusano Milanino, Milan, Italy.

PMID: 34740852 DOI: 10.1016/j.autrev.2021.102990

Abstract

Anti-phospholipid antibodies (aPL) are the serological biomarkers of anti-phospholipid syndrome (APS), an autoimmune disorder characterized by vascular events and/or pregnancy morbidity. APS is a unique condition as thrombosis might occur in arterial, venous or capillary circulations. The heart provides a frequent target for circulating aPL, leading to a wide variety of clinical manifestations. The most common cardiac presentation in APS, valvular involvement, acknowledges a dual etiology comprising both microthrombotic and inflammatory mechanisms. We describe the cases of 4 patients with primary APS who presented a clinically manifest myocardiopathy without epicardial macrovascular distribution. We propose that microthrombotic/inflammatory myocardiopathy might be an overlooked complication of high-risk APS. As extensively hereby reviewed, the literature provides support to this hypothesis in terms of anecdotal case-reports, in some cases with myocardial bioptic specimens. In aPL-positive subjects, microthrombotic/inflammatory myocardial involvement might also clinically manifest as dilated cardiomyopathy, a clinical entity characterized by ventricular dilation and reduced cardiac output. Furthermore, microthrombotic/inflammatory myocardial involvement might be subclinical, presenting as diastolic dysfunction. Currently, there is no single clinical or imaging finding to firmly confirm the diagnosis; an integrated approach including clinical history, clinical assessment, laboratory tests and cardiac magnetic resonance should be pursued in patients with suggestive clinical presentation.

Copyright © 2021. Published by Elsevier B.V.

Keywords: Anti-phospholipid antibodies; Dilated cardiomyopathy; Heart involvement; Inflammation; Myocardium; Systolic dysfunction; Thrombosis

Conflict of interest statement

Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this pa

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