PDF(Pubmed)
Abstract：
OBJECTIVE: 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.
METHODS: 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.
RESULTS: 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%).
CONCLUSIONS: 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.
METHODS: 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.
RESULTS: 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%).
CONCLUSIONS: 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.
摘要：
目的：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。我们报告了极为罕见的SLE病例，伴有严重的弥漫性心肌肥大。
方法：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。排除冠状动脉粥样硬化后，高血压性心肌病,药物毒性，和其他原因，患者被诊断为SLE特异性心肌病.激素等药物，抗疟药,免疫抑制剂,和生物制剂的管理。
结果：辅助检测结果如下：hs-cTnI：0.054ng/mL（0-0.016）；NTproBNP：1594.0pg/mL（<150）；对比增强CMR显示左心室壁弥漫性增厚，并有多个异常增强，左心室收缩和舒张功能降低，和中度心包积液。进行心内膜心肌活检,显示心肌细胞肥大和变性，心肌炎或淀粉样变性没有改变.电镜病理显示心肌细胞内糖原增多,心肌中没有羟氯喹相关的损伤。冠状动脉的24小时动态血压和对比增强计算机断层扫描正常。SLE特异性心肌病的诊断明确。大剂量皮质类固醇治疗后，心肌肥大表现出可逆的缓解。治疗前后CMR结果如下：室间隔，治疗前（28）与治疗后（22）毫米；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％）。
结论：心肌肥厚可能是SLE诊断和治疗中积极和预后评估的重要标志。同样，当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。
方法：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。排除冠状动脉粥样硬化后，高血压性心肌病,药物毒性，和其他原因，患者被诊断为SLE特异性心肌病.激素等药物，抗疟药,免疫抑制剂,和生物制剂的管理。
结果：辅助检测结果如下：hs-cTnI：0.054ng/mL（0-0.016）；NTproBNP：1594.0pg/mL（<150）；对比增强CMR显示左心室壁弥漫性增厚，并有多个异常增强，左心室收缩和舒张功能降低，和中度心包积液。进行心内膜心肌活检,显示心肌细胞肥大和变性，心肌炎或淀粉样变性没有改变.电镜病理显示心肌细胞内糖原增多,心肌中没有羟氯喹相关的损伤。冠状动脉的24小时动态血压和对比增强计算机断层扫描正常。SLE特异性心肌病的诊断明确。大剂量皮质类固醇治疗后，心肌肥大表现出可逆的缓解。治疗前后CMR结果如下：室间隔，治疗前（28）与治疗后（22）毫米；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％）。
结论：心肌肥厚可能是SLE诊断和治疗中积极和预后评估的重要标志。同样，当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。
