Arrhythmogenic cardiomyopathy (AC) is an inherited disease characterized by progressive breakdown of heart muscle, myocardial tissue death, and fibrofatty replacement. In most cases of AC, the primary lesion occurs in one of the genes encoding desmosomal proteins, disruption of which increases membrane fragility at the intercalated disc. Disrupted, exposed desmosomal proteins also serve as epitopes that can trigger an autoimmune reaction. Damage to cell membranes and autoimmunity provoke myocardial inflammation, a key feature in early stages of the disease. In several preclinical models, targeting inflammation has been shown to blunt disease progression, but translation to the clinic has been sparse. Here we review current understanding of inflammatory pathways and how they interact with injured tissue and the immune system in AC. We further discuss the potential role of immunomodulatory therapies in AC.

致心律失常性心肌病 (AC) 是一种遗传性疾病，其特征是心肌进行性分解、心肌组织死亡和纤维脂肪替代。在大多数 AC 病例中，原发性病变发生在编码桥粒蛋白的基因之一中，其破坏会增加闰盘处的膜脆性。破坏的、暴露的桥粒蛋白也可作为触发自身免疫反应的表位。细胞膜和自身免疫的损伤会引发心肌炎症，这是疾病早期的一个关键特征。在几个临床前模型中，靶向炎症已被证明可以减缓疾病进展，但向临床的转化却很少。在这里，我们回顾了目前对炎症通路的理解，以及它们如何与 AC 中受损组织和免疫系统相互作用。