胸腺瘤相关重症肌无力合并炎性肌病三例报道并文献复习  

The coexistence of myasthenia gravis,inflammatory myopathy in patients with thymoma——Three case reports and literature review

作　　者：蒋云[1] 侯世芳[1] 张华[1] 苏闻[1] 张劲松[2] 国红[1] JIANG Yun;HOU Shifang;ZHANG Hua;SU Wen;ZHANG Jinsong;GUO Hong(Department of Neurology,Beijing Hospital,National Center of Gerontology,Institute of Geriatric Medicine,Chinese Academy of Medical Sciences,Beijing 100730,China;不详)

机构地区：[1]北京医院神经内科、国家老年医学中心、中国医学科学院老年医学研究院,100730 [2]北京医院病理科、国家老年医学中心、中国医学科学院老年医学研究院,100730

出　　处：《中国神经免疫学和神经病学杂志》2024年第1期1-7,共7页Chinese Journal of Neuroimmunology and Neurology

基　　金：国家重大疾病多学科合作诊疗能力建设项目基金资助(编号:FTJ-19-03)。

摘　　要：目的分析重症肌无力(myasthenia gravis,MG)合并炎性肌病(inflammatory myopathy,IM)患者的临床特点及其与胸腺瘤的相关性。方法分析北京医院2010-3-1—2020-3-31收治的3例MG合并IM患者的临床特点,并结合文献进行复习。结果3例MG-IM中1例合并谷氨酸脱羧酶抗体阳性的僵肢综合征(stiff limb syndrome,SLS)。3例患者均有胸腺瘤,血清肌酸激酶和肌酸激酶同工酶升高,横纹肌抗体和心肌抗体阳性,而肌炎特异性抗体(myositis specific antibodies,MSA)和肌炎相关抗体(myositis associated antibodies,MAA)均阴性;肌电图提示肌源性损害和神经肌肉接头受累;心电图和超声心动图提示心肌受累;肌肉病理诊断多发性肌炎。联合46篇文献中的93例患者,共96例MG-IM患者纳入汇总分析。MG与IM同时发生者占42.7%,以MG症状首发者占34.4%,以IM症状首发者占22.9%。EMG检查提示肌源性损害和神经肌肉接头突触后膜疾病。乙酰胆碱受体(acetylcholine receptor,AChR)抗体阳性者占91.3%(84/92),MSA抗体阳性者仅占3.0%(2/66),MAA抗体阳性者仅占6.3%(2/32),横纹肌抗体阳性者占95.2%(20/21),6例行心肌抗体检测者均呈阳性。CT检查发现胸腺瘤者占64.4%(58/90)。53例行胸腺瘤手术的患者中,47例描述了胸腺瘤病理分型,以胸腺瘤B2型最常见(19例,40.4%),其次为B1型10例(21.3%),B3型9例(19.1%),AB型8例(17.0%),C型1例(2.1%)。89例行骨骼肌病理检查,其中以多发性肌炎最常见(62例,69.7%),其次为皮肌炎13例(14.6%),肉芽肿性肌炎9例(10.1%),免疫坏死性肌病2例(2.2%),嗜酸性粒细胞肌炎2例(2.2%),包涵体肌炎1例(1.1%)。7例尸体解剖患者行心肌病理检查,均诊断巨细胞性心肌炎。结论MG-IM多见于胸腺瘤患者,部分MG-IM患者同时合并心肌炎。大多数MG-IM患者血MSA和MAA抗体阴性。血横纹肌抗体和心肌抗体阳性提示骨骼肌和心肌受累,巨细胞性心肌炎是MG-IM患者死亡的主要原因之一。MG、IM和SLS全面准确的�Objective To analyze the overlap clinical manifestations of myasthenia gravis(MG)and inflammatory myopathy(IM),and their relationship with thymoma.Methods We performed a retrospective study of MG patients with IM from March 1,2010 to March 31,2020 in Beijing Hospital.Meanwhile,the open published literatures concerning MG-IM were collected.Results Three MG-IM patients were identified and one of them was concurrent with stiff limb syndrome(SLS)associated with elevated glutamic acid decarboxylase antibody.All the 3 patients had thymoma,and presented with increased serum levels of CK,CK-MB,positive anti-striated antibody and anti-myocardium antibody,but negative muscle specific antibodies(MSA)and negative muscle associated(MAA)antibodies.Electromyography(EMG)indicated irritable myopathy and nerve-muscle junction(NMJ)disorders.Electrocardiogram and echocardiograph suggested the probability of myocarditis.Polymyositis was confirmed by muscle biopsy.Combined with 93 MG-IM cases in 46 previous publications,96 cases were analyzed together.IM and MG occurred nearly concurrently in 42.7%cases,MG occurred prior to IM in 34.4%,and IM prior to MG in 22.9%cases.EMG indicated myopathy and NMJ disorders in all the cases.Serum acetylcholine receptor(AChR)antibody was positive in 91.3%(84/92)cases.Serum MSA were positive in only 3.0%(2/66)cases,and serum MAA were positive in only 6.3%(2/32)cases.Anti-striated antibody was positive in 95.2%(20/21)cases.Anti-myocardium antibody was positive in all the 6 detected cases.Chest CT scan revealed thymoma in 64.4%(58/90)cases.Among them,53 cases performed thymectomy and thymoma pathological classification was described in 47 cases.The most common type of thymoma was B2(19 cases,40.4%),followed by 10(21.3%)cases of B1,9(19.1%)cases of B3,8(17.0%)cases of AB,and 1(2.1%)case of type C.In the muscle biopsy of 89 cases,the most common was polymyositis(62 cases,69.7%),followed by 13(14.6%)cases of dermatomyositis,9(10.1%)cases of granulomatous myositis,2(2.2%)cases of necrotizing autoimmune myopa

关 键 词：重症肌无力 炎性肌病 僵肢综合征 胸腺瘤 副肿瘤综合征 肌炎 

分 类 号：R746.1[医药卫生—神经病学与精神病学] R746.9[医药卫生—临床医学]