The spectrum of lupus myocarditis: from asymptomatic forms to cardiogenic shock
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The spectrum of lupus myocarditis: from asymptomatic forms to cardiogenic shock Maya Guglin1 · Carson Smith1 · Roopa Rao1 Accepted: 9 November 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Lupus myocarditis is a serious, potentially deadly disease. When it presents as an acute or fulminant myocarditis in a patient without an established diagnosis of lupus, lupus as an etiology of the condition is not commonly suspected. Meanwhile, it has a distinct treatment which may be lifesaving. Review of the literature can shed more light as current management is mostly based on clinical experience and case reports rather than randomized control trials. In this review we are discussing this diagnostic entity, focusing on cardiogenic shock as a manifestation of lupus myocarditis, and discussing management including aggressive immunosuppression, mechanical circulatory support, and cardiac transplantation. Keywords Lupus myocarditis · Cardiogenic shock · Systemic lupus erythematosus
Introduction
Methods
Systemic lupus erythematosus (SLE) is an immune complex–mediated process that leads to complement activation, inflammation, and myocardial injury. Historically, lupus myocarditis was the last recognized cardiac manifestation of lupus, after the discovery of a non-bacterial verrucous endocarditis by Liebman and Sacks in 1924, and the recognition of pericarditis as a part of lupus polyserositis. Pericardial effusion on echocardiography is present in up to 75% cases [1]. Out of all cardiac manifestations, only pericarditis is included in the diagnostic criteria for SLE [2]. Nevertheless, from a clinical standpoint, myocardial involvement may be more significant as both a prognostic sign and therapeutic challenge. This is especially relevant as the literature has shown that myocarditis is a negative prognosticator for SLE mortality [3, 4]. In this article, we review the current status of diagnosis and management of lupus myocarditis focusing on acute presentation requiring immediate aggressive treatment and support. Lastly, we also discuss the role of potential cardiac transplantation in this condition.
We conducted a systematic search of the databases of PubMed/Medline, Ovid/Medline, Cochrane Library, and Google Scholar, as well as a manual search of references of the relevant papers from January 1990 to June 2020. Each of the co-authors did an independent search. We searched for the following terms: “lupus myocarditis,” as well as the combinations of the following: “lupus” or “systemic lupus erythematosus” and “cardiogenic shock” or “heart transplantation” or “cardiac transplantation,” and “cardiogenic shock.” For the “cardiogenic shock” section we selected the cases where information on individual patient was sufficiently presented, and where the patient required either inotropes/vasopressors or mechanical circulatory support or both.
* Maya Guglin [email protected] 1
Indiana University School of Medicine, Krannert Institute of Cardiology, 1801 Senate Blvd Suite 2000, Indianapolis,
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