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作 者:杨梦婷 赵亚雯 王怡康 袁婧楚 邓建文 刘靖[1] 郑艺明[1] 张巍[2] 王朝霞[2] 袁云[2] Yang Mengting;Zhao Yawen;Wang Yikang;Yuan Jingchu;Deng Jianwen;Liu Jing;Zheng Yiming;Zhang Wei;Wang Zhaoxia;Yuan Yun(Department of Neurology,Peking University First Hospital,Beijing 100034,China;Department of Neurology,Peking University First Hospital,Bejing Key Laboratory of Neurovascular Disease,Beijing 100034,China)
机构地区:[1]北京大学第一医院神经内科,北京100034 [2]北京大学第一医院神经内科、北京市神经血管病探索重点实验室,北京100034
出 处:《中华神经科杂志》2024年第1期40-46,共7页Chinese Journal of Neurology
基 金:国家自然科学基金(82201550)。
摘 要:目的探讨新型冠状病毒(SARS-CoV-2)感染(COVID-19)相关免疫性肌病的临床、影像、肌肉病理改变特点和治疗转归。方法收集2022年12月至2023年4月在北京大学第一医院确诊的5例SARS-CoV-2感染后免疫性肌病患者的临床特征、血清肌酸激酶、肌炎抗体、肌肉磁共振成像、肌肉病理资料,短期随访后分析其治疗效果。结果5例患者中,男性3例,女性2例,发病年龄42~86岁,均表现为COVID-19症状消失后不久出现肢体近端无力,其中3例伴肌痛、1例伴吞咽困难、2例伴皮肤损害、1例伴间质性肺病。5例的血清肌酸激酶在1663~16000 IU/L,分别有1例出现抗3-羟基-3甲基戊二酰辅酶A还原酶抗体和抗信号识别颗粒抗体,肌电图均显示肌源性损害,肌肉磁共振成像表现为弥漫性肌肉水肿,3例伴皮下组织和肌筋膜水肿。4例肌肉活组织检查结果提示坏死性肌病,伴随肌纤维内P62阳性沉积。2例电镜检查可见肌纤维出现线粒体空泡化以及核内管丝样包涵体。4例使用糖皮质激素,其中2例联合使用丙种球蛋白、环磷酰胺或他克莫司,1例采用观望疗法,结果肌无力均有不同程度好转,但4例患者的血清肌酸激酶依然高于正常水平。结论COVID-19可以引起免疫性肌病,主要表现为肢体近端无力伴血清肌酸激酶增高,肌肉磁共振成像可见弥漫性水肿,肌肉活组织检查显示坏死性肌病,免疫抑制治疗有效性还需要继续观察。Objective To analyze the clinical characteristics,imaging,myopathology and outcomes of patients with COVID-19 related autoimmune myopathy.Methods The clinical features,serum creatine kinase(CK),myositis antibodies,muscle magnetic resonance imaging,myopathology and therapy of 5 patients with COVID-19 related autoimmune myopathy diagnosed in Peking University First Hospital from December 2022 to April 2023 were collected.The effects of the therapy after a short term follow up were analyzed.Results Among the 5 patients,there were 3 males and 2 females,with onset age of 42-86 years.All patients presented with proximal muscle weakness in the recovery term of COVID-19.Myalgia was noted in 3 cases,dysphagia in 1,skin damage in 2,interstitial lung disease in 1.The serum CK of the 5 patients was 1663-16000 IU/L,1 patient had anti-3-hydroxy-3-methylglutaryl-CoA reductase autoantibodies and 1 patient had anti-signal recognition particle autoantibodies.The electromyography showed myogenic lesions in all patients.Muscle magnetic resonance imaging showed diffuse muscle edema in all patients,myofascial edema in 3 and subcutaneous-tissue edema in 3.The muscle biopsies in 4 patients revealed necrotic myopathy,with high P62 expression in muscle fibers.The electromicroscopy of 2 patients revealed vacuolated mitochondria and intranuclear tubulofilamentous inclusions in muscle fibers.Four patients were treated with glucocorticoids,of whom 2 patients combined with intravenous immunoglobulin,tacrolimus or cyclophosphamide.One case had close monitoring without drug therapy.They showed significant improvement,but the CK was still abnormal in 4 patients.Conclusions COVID-19 leads to immune mediated myopathy.The manifestation of patients is characterized by proximal predominant weakness and high creatine kinase level.Muscle magnetic resonance imaging shows diffuse muscle edema.The muscle biopsies reveal necrotic myopathy.The effectiveness of immunosuppression needs to be further studied.
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