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
蒋云 1侯世芳 1张华 1苏闻 1张劲松 2国红1
作者信息
- 1. 100730 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
- 2. 100730 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
- 折叠
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全面准确的诊断依赖于临床、电生理、免疫学和肌肉病理的综合评估.
Abstract
Objective T o 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 myopathy,2(2.2％)cases of eosinophilic myositis,and 1(1.1％)case of inclusion body myositis.Myocardial histology indicated giant cell myocarditis in all the 7 autopsy cases.Conclusions MG-IM was mostly occurred in patients with thymoma,and some of them had myocarditis.Majority of MG-IM patients were negative of either MSA or MAA.The presence of anti-striated antibody or anti-myocardium antibody indicated the involvement of skeletal muscles or myocardium.Giant cell myocarditis increased the mortality risk.The comprehensive diagnosis of MG,IM,as well as SLS,was dependent on the careful evaluations of clinical features,electrophysiology,immunology,and muscle biopsy.
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Key words
myasthenia gravis/inflammatory myopathy/stiff limb syndrome/thymoma/paraneoplastic syndrome/myositis引用本文复制引用
基金项目
国家重大疾病多学科合作诊疗能力建设项目(FTJ-19-03)
出版年
2024
NETL
NSTL
维普
万方数据