Hamana Tomoyo, Kawamori Hiroyuki, Satomi-Kobayashi Seimi, Yamamoto Yuzuru, Ikeda Yoshihiko, Hirata Ken-Ichi
Division of Cardiovascular Medicine, Department of Internal Medicine, Kobe University Graduate School of Medicine, 7-5-1 Kusunoki-cho, Chuo-ku, Kobe, Hyogo 650-0017, Japan.
Division of Rheumatology and Clinical Immunology, Department of Internal Medicine, Kobe University Graduate School of Medicine, Kobe, Japan.
Eur Heart J Case Rep. 2023 Apr 10;7(4):ytad174. doi: 10.1093/ehjcr/ytad174. eCollection 2023 Apr.
Although cardiac involvement is relatively common in mixed connective tissue disease (MCTD), few reports on MCTD-associated fulminant myocarditis are available.
A 22-year-old woman diagnosed with MCTD was admitted to our institution for cold-like symptoms and chest pain. Echocardiography revealed that the left ventricular ejection fraction (LVEF) had rapidly decreased from 50 to 20%. Because endomyocardial biopsy revealed no significant lymphocytic infiltration, immunosuppressant drugs were not started initially; however, steroid pulse therapy (methylprednisolone, one1000 mg/day) was initiated due to prolonged symptoms and unimproved haemodynamics. Despite strong immunosuppressant therapy, the LVEF did not improve, and severe mitral regurgitation appeared. Three days after steroid pulse therapy initiation, she experienced a sudden cardiac arrest; thus, venoarterial extracorporeal membrane oxygenation (VA-ECMO) and intra-aortic balloon pumping (IABP) were initiated. Subsequent immunosuppressant therapy was continued with prednisolone (100 mg/day) and intravenous cyclophosphamide (1000 mg). Six days after steroid therapy initiation, the LVEF improved to 40% and then recovered to near-normal levels. After successful weaning off of VA-ECMO and IABP, she was discharged. Thereafter, a detailed histopathological examination revealed multi-focal signs of ischaemic micro-circulatory injury and diffuse HLA-DR in the vascular endothelium, suggesting an autoimmune inflammatory response.
We report a rare case of fulminant myocarditis in a patient with MCTD who recovered with immunosuppressive treatment. Despite the absence of significant lymphocytic infiltration findings on histopathological examination, patients with MCTD may experience a dramatic clinical course. Although it is unclear whether myocarditis is triggered by viral infections, certain autoimmune mechanisms may lead to its development.
尽管心脏受累在混合性结缔组织病（MCTD）中相对常见，但关于MCTD相关暴发性心肌炎的报道较少。
一名22岁诊断为MCTD的女性因类似感冒症状和胸痛入住我院。超声心动图显示左心室射血分数（LVEF）迅速从50%降至20%。由于心内膜活检未发现明显淋巴细胞浸润，最初未开始使用免疫抑制药物；然而，由于症状持续且血流动力学未改善，开始使用类固醇脉冲疗法（甲泼尼龙，1000mg/天）。尽管进行了强力免疫抑制治疗，LVEF仍未改善，且出现了严重二尖瓣反流。在开始类固醇脉冲治疗三天后，她突然心脏骤停；因此，启动了静脉-动脉体外膜肺氧合（VA-ECMO）和主动脉内球囊反搏（IABP）。随后继续使用泼尼松龙（100mg/天）和静脉注射环磷酰胺（1000mg）进行免疫抑制治疗。在开始类固醇治疗六天后，LVEF改善至40%，然后恢复到接近正常水平。成功撤掉VA-ECMO和IABP后，她出院了。此后，详细的组织病理学检查显示存在多灶性缺血性微循环损伤迹象以及血管内皮弥漫性HLA-DR表达，提示自身免疫性炎症反应。
我们报告了一例MCTD患者发生暴发性心肌炎并通过免疫抑制治疗康复的罕见病例。尽管组织病理学检查未发现明显淋巴细胞浸润，但MCTD患者可能经历戏剧性的临床病程。虽然尚不清楚心肌炎是否由病毒感染引发，但某些自身免疫机制可能导致其发生。
