1. Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies.
- Author
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Gasperetti A, Carrick RT, Muller S, Murray B, Adamo L, Bauce B, McNally E, and Helms A
- Subjects
- Humans, Immunosuppressive Agents therapeutic use, Anti-Inflammatory Agents therapeutic use, Inflammation drug therapy, Myocarditis drug therapy, Desmoplakins, Cardiomyopathies drug therapy
- Abstract
Purpose of Review: To summarize the available data on the use of immunosuppression therapies for the management of hot phases of disease and recurrent myocarditis in patients with desmoplakin cardiomyopathy (DSP-CMP)., Recent Findings: Occurrence of myocarditis episodes has been associated with worsening of outcomes in DSP-CMP. Multiple case reports and small case series have described potential benefit in using anti-inflammatory and immunosuppressive medications for the treatment of those episodes. Recently published translational data has shown a clear link between DSP variants and a propensity to inflammation. The presence of acute myocarditis episodes have been clearly demonstrated as a manifestation of DSP-CMP. These episodes are marked by myocardial inflammation and subsequent fibrosis, and, moreover, contribute to a heightened future risk of subsequent arrhythmias and heart failure. Identifying optimal strategies to prevent and/or interrupt myocardial inflammation is therefore a critical priority of patients with DSP-CMP., Competing Interests: Declarations. Conflict of Interest: The authors declare no competing interests. Human and Animal Rights and Informed Consent: This article does not contain any studies with human or animal subjects performed by any of the authors., (© 2025. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)
- Published
- 2025
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