Williams Carson L, Dean John-Henry L, Patel Mayank, Cahill Michael S, Kunavarapu Chandra, Kwan Michael
Internal Medicine, San Antonio Uniformed Services Health Education Consortium, San Antonio, USA.
Cardiology, San Antonio Uniformed Services Health Education Consortium, San Antonio, USA.
Cureus. 2024 Oct 22;16(10):e72152. doi: 10.7759/cureus.72152. eCollection 2024 Oct.
Idiopathic inflammatory myopathies (IIM) are known to have extramuscular involvement, the most concerning of which is the involvement of the myocardium. Constituting a large burden of morbidity and mortality, there remains a paucity of literature describing cardiac manifestations in inflammatory myopathies, and definitive treatment and screening guidelines have yet to be published. Here, we present a rare case of cardiac arrest and fulminant myocarditis in a patient with newly diagnosed myositis. A 71-year-old non-Hispanic White male with type 1 diabetes mellitus and hyperlipidemia presented to the rheumatology clinic with five months of progressive proximal muscle weakness and myalgias accompanied by a persistently elevated creatine kinase level and elevated liver-associated enzymes despite cessation of atorvastatin therapy three months prior. The initial examination was notable for reduced quadriceps strength bilaterally and the absence of visible skin rashes. He was found to have positive anti-Mi-2 antibody, elevated aldolase, and positive antinuclear antibody in a speckled pattern. After magnetic resonance imaging (MRI) of the left thigh demonstrated a pattern consistent with inflammatory myositis, steroid therapy was initiated, and he was referred for muscle biopsy to confirm the presumptive diagnosis of dermatomyositis. Two weeks later, before a muscle biopsy could be performed, the patient experienced a witnessed pulseless electrical activity (PEA) cardiac arrest from which he was successfully resuscitated by emergency medical services prior to hospital arrival. Subsequent cardiac evaluation showed a nonischemic cardiomyopathy with evidence of myocarditis on cardiac magnetic resonance (CMR) imaging with inferior wall hypokinesis and a left ventricular ejection fraction (LVEF) of 27%. He underwent placement of a subcutaneous implantable cardioverter defibrillator (ICD), and he responded well to intravenous immunoglobulin (IVIG), diuresis, and initiation of guideline-directed medical therapy with post-treatment transthoracic echocardiogram (TTE) demonstrating an LVEF of 40%. This case highlights one of the rather protean and severe cardiac manifestations of IIM. Typically, the cardiac manifestations observed in IIM include subclinical electrocardiogram (ECG) and echocardiographic changes but can present, as detailed here, with fulminant myocarditis and heart failure (HF). Our purpose herein is to heighten clinician awareness of and advocate for the establishment of definitive screening and management guidelines for cardiac disease in idiopathic inflammatory myopathies.
特发性炎性肌病（IIM）已知会出现肌肉外受累，其中最令人担忧的是心肌受累。炎性肌病的心脏表现相关文献较少，且明确的治疗和筛查指南尚未发布，但其构成了较大的发病和死亡负担。在此，我们报告一例新诊断的肌炎患者发生心脏骤停和暴发性心肌炎的罕见病例。一名71岁非西班牙裔白人男性，患有1型糖尿病和高脂血症，因进行性近端肌无力和肌痛5个月就诊于风湿病诊所，尽管3个月前已停用阿托伐他汀治疗，但肌酸激酶水平持续升高，且肝脏相关酶也升高。初次检查发现双侧股四头肌力量减弱，无明显皮疹。他抗Mi-2抗体阳性、醛缩酶升高，抗核抗体斑点型阳性。左大腿磁共振成像（MRI）显示符合炎性肌炎的表现后，开始使用类固醇治疗，并转诊进行肌肉活检以确诊皮肌炎的初步诊断。两周后，在进行肌肉活检之前，患者出现目击的无脉电活动（PEA）心脏骤停，在到达医院之前，紧急医疗服务成功将其复苏。随后的心脏评估显示为非缺血性心肌病，心脏磁共振（CMR）成像显示有心肌炎证据，下壁运动减弱，左心室射血分数（LVEF）为27%。他接受了皮下植入式心律转复除颤器（ICD）植入，对静脉注射免疫球蛋白（IVIG）、利尿治疗以及开始遵循指南的药物治疗反应良好，治疗后的经胸超声心动图（TTE）显示LVEF为40%。该病例突出了IIM相当多变且严重的心脏表现之一。通常，IIM中观察到的心脏表现包括亚临床心电图（ECG）和超声心动图改变，但也可能如本文详述的那样，表现为暴发性心肌炎和心力衰竭（HF）。我们的目的是提高临床医生对特发性炎性肌病中心脏疾病的认识，并倡导建立明确的筛查和管理指南。
