抗信号识别颗粒抗体阳性的免疫介导坏死性肌病的临床、肌肉影像及病理特点分析  

Clinical manifestation,muscular imaging and pathological characteristics of anti-signal recognition particle positive immune-mediated necrotizing myopathy

作　　者：李诗瑶 瞿千千 郑献召 马晓丽 崔文豪 李丹[2] 吕正 赵炯博 王佳璇 胡聪 吕海东 Li Shiyao;Qu Qianqian;Zheng Xianzhao;Ma Xiaoli;Cui Wenhao;Li Dan;Lyu Zheng;Zhao Jiongbo;Wang Jiaxuan;Hu Cong;Lyu Haidong(Department of Neurology,Jiaozuo People's Hospital Affiliated to Xinxiang Medical College,Jiaozuo 454002,China;Department of Neuroimaging,Jiaozuo People's Hospital Affiliated to Xinxiang Medical College,Jiaozuo 454002,China)

机构地区：[1]新乡医学院附属焦作市人民医院神经内科,焦作454002 [2]新乡医学院附属焦作市人民医院神经影像科,焦作454002

出　　处：《中华神经科杂志》2024年第9期1000-1008,共9页Chinese Journal of Neurology

基　　金：河南省医学科技攻关计划(LHGJ20191340)。

摘　　要：目的:分析抗信号识别颗粒抗体阳性的免疫介导坏死性肌病(SRP-IMNM)患者的临床、肌肉影像及病理特点。方法:收集2018年5月至2023年5月就诊于焦作市人民医院神经肌肉病中心、经骨骼肌病理和肌炎特异性抗体(MSAs)确诊的9例SRP-IMNM患者的临床资料,分析并总结其临床特征、肌肉影像学及肌肉病理学特点。结果:9例患者中女性7例,男性2例,起病年龄为18~59岁。9例患者临床均表现为四肢近端肌无力,7例伴颈肌无力,5例有吞咽困难。9例患者的血清肌酸激酶均有不同程度升高(1 866~6 725 U/L)。其中4例合并间质性肺病,3例合并心脏受累。抗体检测9例患者除SRP抗体阳性以外,有8例合并其他抗体阳性,7例合并抗Ro-52抗体阳性,4例为单纯合并抗Ro-52抗体阳性,3例同时合并3种以上抗体阳性。其中出现间质性肺病和心脏受累的患者均合并其他抗体阳性。7例患者行大腿肌肉磁共振成像(MRI)检查均可见弥漫性骨骼肌水肿,部分肌肉萎缩及脂肪化,病变主要累及大腿后侧肌群。有2例患者行小腿肌肉MRI,可见比目鱼肌受累较明显,1例有胫骨前肌、腓肠肌受累。9例患者肌肉病理均可见有不同程度的肌纤维变性、坏死和再生,1例可见少量炎性细胞浸润。4例患者可见管柱状毛细血管。免疫组织化学染色可见8例患者坏死肌纤维内有少量CD68阳性淋巴细胞,5例患者可见肌纤维膜上有主要组织相容性复合体Ⅰ表达上调,6例患者非坏死肌纤维和毛细血管上可见膜攻击复合物(C5b-9)沉积,6例患者行P62染色均可见部分肌纤维内有均质的细颗粒样物质沉积。结论:SRP-IMNM患者除肢体近端肌无力外,常伴有颈肌无力和吞咽困难,合并多种抗体阳性的患者更容易出现间质性肺病和心脏受累。SRP-IMNM患者受累肌肉呈弥漫性水肿,大腿后侧肌群更易发生萎缩和脂肪化。C5b-9沉积和管柱状毛细血管是SRP-IMNM的重要病理特�Objective:To analyze the clinical characteristics,muscle imaging and pathological features of patients with anti-signal recognition particle positive immune-mediated necrotizing myopathy(SRP-IMNM).Methods:Nine patients with SRP-IMNM were collected in the Neuromuscular Disease Center of Jiaozuo People′s Hospital from May 2018 to May 2023,who were confirmed by skeletal muscle pathology and myositis-specific autoantibodies detection,and their clinical manifestations,muscle imaging and muscle pathology characteristics were systematically summarized.Results:Among the 9 patients with SRP-IMNM,there were 7 females and 2 males.The age of onset ranged from 18 to 59 years.All the patients presented proximal muscle weakness.Seven patients experienced neck weakness,and dysphagia was present in 5 patients.Laboratory examinations showed elevated serum creatine kinase levels in all 9 patients(1866-6725 U/L).Eight patients were combined with other antibodies positivity,except for anti-SRP antibody.Among them,7 patients were combined with anti-Ro-52 antibody positivity,4 patients combined with anti-Ro-52 antibody positivity alone,and 3 patients combined with 3 or more positive antibodies simultaneously.Those patients who presented with interstitial lung disease and cardiac involvement were all combined with other antibodies positivity.Seven patients completed thigh muscle magnetic resonance imaging(MRI),which showed diffuse skeletal muscle oedema,partial muscle atrophy and fatty replacement,primarily affecting the posterior thigh muscle group.Two patients underwent shank muscle MRI.The soleus involvement was evident,while the tibialis anterior muscle and gastrocnemius muscles were involved in 1 patient.All 9 patients showed varying degrees of scattered muscle fiber necrosis and regeneration on muscle biopsies.In 1 patient,a small amount of inflammatory cell infiltration was observed.Pipestem capillaries were observed in 4 patients.Immunohistochemical staining revealed a small number of CD68-positive lymphocytes in 8 patients.Ad

关 键 词：信号识别颗粒 免疫介导坏死性肌病 磁共振成像 肌肉病理 

分 类 号：R685[医药卫生—骨科学]