Desmoplakin Cardiomyopathy: Role of Inflammation and Potential Role of Disease-Modifying Therapies.

IF 3.1 3区医学 Q2 CARDIAC & CARDIOVASCULAR SYSTEMS Current Cardiology Reports Pub Date : 2025-01-09 DOI:10.1007/s11886-024-02183-7

Alessio Gasperetti, Richard T Carrick, Steven Muller, Brittney Murray, Luigi Adamo, Barbara Bauce, Elizabeth McNally, Adam Helms

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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.

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Desmoplakin心肌病：炎症的作用和疾病改善治疗的潜在作用。

综述目的：总结利用免疫抑制疗法治疗desmoplakin心肌病（DSP-CMP）患者疾病热期和复发性心肌炎的现有资料。最近发现：心肌炎发作的发生与spd - cmp预后的恶化有关。多个病例报告和小病例系列描述了使用抗炎和免疫抑制药物治疗这些发作的潜在益处。最近发表的翻译数据显示了DSP变异和炎症倾向之间的明确联系。急性心肌炎发作的存在已被清楚地证明是DSP-CMP的表现。这些发作的标志是心肌炎症和随后的纤维化，而且，有助于提高未来发生心律失常和心力衰竭的风险。因此，确定预防和/或中断心肌炎症的最佳策略是DSP-CMP患者的关键优先事项。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Current Cardiology Reports CARDIAC & CARDIOVASCULAR SYSTEMS-

CiteScore

6.20

自引率

2.70%

发文量

209

期刊介绍： The aim of this journal is to provide timely perspectives from experts on current advances in cardiovascular medicine. We also seek to provide reviews that highlight the most important recently published papers selected from the wealth of available cardiovascular literature. We accomplish this aim by appointing key authorities in major subject areas across the discipline. Section editors select topics to be reviewed by leading experts who emphasize recent developments and highlight important papers published over the past year. An Editorial Board of internationally diverse members suggests topics of special interest to their country/region and ensures that topics are current and include emerging research. We also provide commentaries from well-known figures in the field.