Innovative Institute of Chinese Medicine and Pharmacy, Shandong University of Traditional Chinese Medicine, Jinan, China.
The Fifth People's Hospital of Jinan, Jinan, China.
Immun Inflamm Dis. 2024 Mar;12(3):e1214. doi: 10.1002/iid3.1214.
Systemic lupus erythematosus (SLE) is a multisystem-involved, highly heterogeneous autoimmune disease with diverse clinical manifestations. We report an extremely rare case of SLE with severe diffuse myocardial hypertrophy.
The patient's echocardiography and cardiac magnetic resonance imaging (CMR) results indicated diffuse myocardial hypertrophy. After excluding coronary atherosclerosis, hypertensive cardiomyopathy, drug toxicity, and other causes, the patient was diagnosed with SLE-specific cardiomyopathy. Medications such as hormones, antimalarials, immunosuppressants, and biologics were administered.
Ancillary test results were as follows: hs-cTnI: 0.054 ng/mL (0-0.016); NTproBNP: 1594.0 pg/mL (<150); A contrast-enhanced CMR revealed the diffuse thickening of the left ventricular wall with multiple abnormal enhancements, reduced left ventricular systolic and diastolic function, and moderate amount of pericardial effusion. Endomyocardial myocardial biopsy was performed, showing cardiomyocyte hypertrophy and degeneration, and no changes in myocarditis or amyloidosis. The pathology viewed by electron microscopy showed increased intracellular glycogen in the myocardium, and no hydroxychloroquine-associated damage in the myocardium. The 24-h ambulatory blood pressure and contrast-enhanced computed tomography of coronary arteries were normal. The diagnosis of SLE-specific cardiomyopathy was clear. The myocardial hypertrophy showed reversible alleviation following treatment with high-dose corticosteroids. CMR results before and after treatment were as follows: interventricular septum, pretreatment (28) versus post-treatment (22) mm; left ventricular inferior wall, pretreatment (18-21) versus post-treatment (12-14) mm; left ventricular lateral wall, pretreatment (17-18) versus post-treatment (10-12) mm; pericardial effusion (left ventricular lateral wall), pretreatment (25) versus post-treatment (12) mm; left ventricular ejection fraction, pretreatment (38.9%) versus post-treatment (66%).
Myocardial hypertrophy may be an important sign of active and prognostic assessment in SLE diagnosis and management. Similarly, when encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to common causes.
系统性红斑狼疮（SLE）是一种多系统受累、高度异质性的自身免疫性疾病，临床表现多样。我们报告一例极罕见的系统性红斑狼疮伴严重弥漫性心肌肥厚病例。
患者的超声心动图和心脏磁共振成像（CMR）结果提示弥漫性心肌肥厚。在排除冠状动脉粥样硬化、高血压性心肌病、药物毒性等原因后，诊断为系统性红斑狼疮特异性心肌病。给予激素、抗疟药、免疫抑制剂和生物制剂等药物治疗。
辅助检查结果如下：超敏肌钙蛋白 I：0.054ng/ml（0-0.016）；N 末端脑利钠肽前体：1594.0pg/ml（＜150）；对比增强 CMR 显示左心室壁弥漫性增厚，多发异常强化，左心室收缩和舒张功能降低，中等量心包积液。行心内膜心肌活检，表现为心肌细胞肥大和变性，无心肌炎或淀粉样变性改变。电镜下病理显示心肌细胞内糖原增多，心肌内无羟氯喹相关损伤。24 小时动态血压和冠状动脉增强 CT 正常。明确诊断为系统性红斑狼疮特异性心肌病。大剂量皮质激素治疗后，心肌肥厚呈可逆性缓解。治疗前后 CMR 结果如下：室间隔，治疗前（28）与治疗后（22）mm；左心室下壁，治疗前（18-21）与治疗后（12-14）mm；左心室侧壁，治疗前（17-18）与治疗后（10-12）mm；心包积液（左心室侧壁），治疗前（25）与治疗后（12）mm；左心室射血分数，治疗前（38.9%）与治疗后（66%）。
心肌肥厚可能是系统性红斑狼疮诊断和治疗中评估活动性和预后的重要指标。同样，在遇到心肌肥厚的病例时，除了常见原因外，还应考虑自身免疫性疾病的可能性。
