Ziegler John P, Batalis Nicholas I, Fulcher James W, Ward Michael E
Medical University of South Carolina, Charleston, SC, USA.
Medical University of South Carolina, Department of Pathology, Charleston, SC, USA.
Autops Case Rep. 2020 Nov 20;10(4):e2020238. doi: 10.4322/acr.2020.238.
Giant cell myocarditis (GCM) is a rare and rapidly fatal cardiovascular condition most often seen in young adults. It is characterized microscopically by myocardial necrosis with multinucleated giant cells in the absence of well-defined granulomas. This disorder has typically been attributed to manifest as heart failure, but in some individuals, GCM may present as sudden cardiac death. Herein, we present a fatal case of GCM in a 36-year-old male with a history of autoimmune disorders. The decedent presented to the emergency room due to vomiting and was treated for nausea due to suspected dehydration. He was discharged that night and found dead on his bathroom floor the following day. Postmortem examination revealed psoriasis and granulomatous lesions in the lungs consistent with sarcoidosis, further supporting circumstantial evidence existing between GCM and autoimmune disorders. Additionally, this case provides an opportunity to distinguish GCM from the distinct clinical entity of cardiac sarcoidosis (CS), especially in the setting of systemic sarcoidosis. We hope to raise awareness of this rare disease process and its potential to cause sudden cardiac death so that it may be considered in a differential diagnosis as immunosuppression and early cardiac transplantation largely determine the prognosis.
巨细胞性心肌炎(GCM)是一种罕见且迅速致命的心血管疾病,多见于年轻人。其微观特征为心肌坏死伴多核巨细胞,且无明确的肉芽肿。这种疾病通常表现为心力衰竭,但在某些个体中,GCM可能表现为心源性猝死。在此,我们报告一例36岁男性GCM致死病例,该患者有自身免疫性疾病史。死者因呕吐就诊于急诊室,因怀疑脱水接受了恶心治疗。当晚出院,次日被发现死于浴室地板上。尸检显示肺部有银屑病和与结节病相符的肉芽肿性病变,进一步支持了GCM与自身免疫性疾病之间存在的间接证据。此外,该病例为区分GCM与心脏结节病(CS)这一不同临床实体提供了机会,尤其是在系统性结节病的背景下。我们希望提高对这种罕见疾病过程及其导致心源性猝死可能性的认识,以便在鉴别诊断中予以考虑,因为免疫抑制和早期心脏移植在很大程度上决定了预后。