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Review
. 2024 Jul 15;12(7):1565.
doi: 10.3390/biomedicines12071565.

Immunomodulating and Immunosuppressive Therapy for Virus-Negative Immune-Mediated Myocarditis

Andrea Frustaci  1   2 , Claudio Letizia  3 , Maria Alfarano  3 , Giulia Marchionni  4 , Romina Verardo  1 , Cristina Chimenti  3
Affiliations
Review

Immunomodulating and Immunosuppressive Therapy for Virus-Negative Immune-Mediated Myocarditis

Andrea Frustaci et al. Biomedicines. .

Abstract

Myocarditis is an inflammatory disease of the myocardium caused by infectious and noninfectious agents. Clinical manifestations range from mildly symptomatic forms to acute heart failure, cardiogenic shock, life-threatening arrhythmias and sudden death. Myocarditis is still a challenging diagnosis because of its wide variability in clinical presentation and unpredictable course. Moreover, a standardized, specific treatment in not yet available. Immunosuppressive treatment for virus-negative lymphocytic myocarditis is still controversial. Conversely, immunosuppression is well established in sarcoidosis, eosinophilic, giant-cell, drug hypersensitivity, and trauma-related myocarditis as well as lymphocytic myocarditis associated with connective tissue diseases or with the rejection of a transplanted heart. Recently, immunosuppressive therapy has been also recognized as an effective treatment in virus-negative inflammatory cardiomyopathy. The aim of this review is to underline the role of immunomodulating and immunosuppressive therapies in patients with immune-mediated myocarditis and illustrate the different treatment strategies depending on the etiology. An endomyocardial biopsy remains the gold standard for the diagnosis of myocarditis as well as for a tailored treatment.

Keywords: autoimmunity; endomyocardial biopsy; immunosuppressive therapy; myocarditis.

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Conflict of interest statement

The authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Major types of myocarditis that are amenable to immune suppressive therapy. (A) Virus-negative lymphocytic myocarditis. H&E 100×. (B) Lymphocytic myocarditis associated with necrotizing coronary vasculitis. Immunostaining with CD45Ro, 400×. (C) Eosinophilic myocarditis. H&E 325×. (D) Giant cell myocarditis. H&E 200×. (E) Hypertrophic cardiomyopathy with over-imposition of lymphocytic myocarditis. H&E 225×. (F) Fabry disease cardiomyopathy with over-imposition of immune-mediated (vs. GB3) myocarditis. Immunostaining with CD45Ro, 400×. Scale bar, 5 microns.
Figure 2
Figure 2
Diagnostic and therapeutic work-up in immune-mediated myocarditis. In cases of suspected clinical myocarditis with uncomplicated presentations, the diagnostic and therapeutic work-up may include cardiac MRI imaging and supportive therapy. On the contrary, in cases of complicated myocarditis, cardiac MRI imaging is useful, but EMB is the gold standard to establish etiology and initiate the most appropriate treatment in association with therapy for heart failure and arrhythmias.
See this image and copyright information in PMC

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