Yudhianto Eric, Malisie Ririe F, Abdillah Hafaz Z, Rusli Rita E
Department of Emergency Medicine, Murni Teguh Memorial Hospital, Medan, Indonesia.
Department of Pediatrics, Murni Teguh Memorial Hospital, Medan, Indonesia.
Narra J. 2025 Apr;5(1):e1621. doi: 10.52225/narra.v5i1.1621. Epub 2025 Feb 17.
Cardiomyopathy is a rare clinical manifestation in pediatric systemic lupus erythematosus (SLE), with only a single case reported in the literature. Its identification in pediatric SLE is challenging due to its typically subclinical presentation and low incidence, which frequently result in delayed diagnosis and management. The aim of this study was to present a unique case of dilated cardiomyopathy, a rare cardiac complication of SLE, which can be life-threatening if not promptly recognized and treated. An 11-year-old boy was admitted to the emergency department of Murni Teguh Memorial Hospital, Medan, Indonesia, diagnosed with SLE based on the 2019 European Alliance of Associations for Rheumatology (EULAR) criteria, with a total score of 30 and a Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) score of 16, indicating high disease activity. Clinical findings included oral ulcers, a non-pruritic hyperpigmented discoid macule, anemia, lymphopenia, positive both the direct and indirect Coombs tests, elevated D-dimer level, and pulmonary congestion. Initial treatment stabilized the patient condition, allowing transfer to the general ward by day five. Five days after admission, the patient developed palpitations and tachycardia, with a heart rate of 140 beats per minute. Electrocardiography showed sinus tachycardia, while echocardiography revealed all cardiac chambers dilation, left ventricular ejection fraction (LVEF) of 43%, moderate mitral regurgitation, and mild pulmonary regurgitation, subsequently diagnosed as dilated cardiomyopathy. Heart failure therapy was initiated with intravenous furosemide, oral ramipril, and digoxin. Palpitations and tachycardia resolved within two days. Following two weeks of treatment, the patient was discharged with stable vital signs. A one-month follow-up thoracic echocardiography demonstrated improved cardiomyopathy, with an LVEF of 53%. Cardiomyopathy in pediatric SLE is rare but can cause significant morbidity and mortality if undiagnosed. Its nonspecific presentation and immune-mediated pathogenesis make early detection challenging. Due to its rarity, it may be overlooked, highlighting the importance of comprehensive cardiac evaluation, including echocardiography, in children with suspected cardiac involvement.
心肌病是儿童系统性红斑狼疮（SLE）中一种罕见的临床表现，文献中仅报道过一例。由于其通常为亚临床症状且发病率低，在儿童SLE中识别心肌病具有挑战性，这常常导致诊断和治疗延迟。本研究的目的是介绍一例扩张型心肌病的独特病例，这是SLE一种罕见的心脏并发症，如果不能及时识别和治疗可能危及生命。一名11岁男孩入住印度尼西亚棉兰穆尔尼·特古纪念医院急诊科，根据2019年欧洲抗风湿病联盟（EULAR）标准被诊断为SLE，总分30分，系统性红斑狼疮疾病活动指数（SLEDAI）评分为16分，表明疾病活动度高。临床发现包括口腔溃疡、非瘙痒性色素沉着盘状斑、贫血、淋巴细胞减少、直接和间接抗人球蛋白试验均阳性、D - 二聚体水平升高以及肺充血。初始治疗使患者病情稳定，到第五天可转至普通病房。入院五天后，患者出现心悸和心动过速，心率为每分钟140次。心电图显示窦性心动过速，而超声心动图显示所有心腔扩张，左心室射血分数（LVEF）为43%，中度二尖瓣反流和轻度肺动脉反流，随后被诊断为扩张型心肌病。开始采用静脉注射呋塞米、口服雷米普利和地高辛进行心力衰竭治疗。心悸和心动过速在两天内缓解。经过两周治疗，患者生命体征稳定出院。一个月的随访胸部超声心动图显示心肌病有所改善，LVEF为53%。儿童SLE中的心肌病罕见，但如果未被诊断，可能导致严重的发病率和死亡率。其非特异性表现和免疫介导的发病机制使得早期检测具有挑战性。由于其罕见性，可能会被忽视，这凸显了对疑似心脏受累儿童进行包括超声心动图在内的全面心脏评估的重要性。
