摘要
目的 归纳抗Mi-2抗体阳性特发性炎性肌病(IIM)患者临床及骨骼肌病理特点。方法 收集2019年1月1日—2022年2月28日在解放军总医院第一医学中心诊治的7例抗Mi-2抗体阳性IIM患者的临床资料进行回顾性分析,并结合国内外文献复习。结果 7例以近端肌无力更多见,可伴吞咽困难,易伴发肌痛、关节痛和肌萎缩。4例出现较重皮疹。伴发间质性肺炎(ILD)较少,可伴发恶性肿瘤或甲状腺功能减退/疫苗接种史等。骨骼肌病理可见炎性肌病的病理改变,符合束周免疫肌病(IMPP)的特点。免疫组织化学染色见人类主要组织相容性复合体MHC-Ⅰ肌内衣表达阳性和抗黏液病毒A蛋白(MxA)束周肌纤维膜和肌浆阳性表达。7例患者使用糖皮质激素和免疫抑制剂联合治疗后3个月,患者情况均较前好转。结论 抗Mi-2抗体阳性IIM患者临床表型多见皮肌炎(DM)经典皮疹、肌痛、肌无力等。肌肉活检可见IMPP,伴ILD较少,可伴免疫性疾病。类固醇联合免疫抑制剂反应较好。
Objective To summarize the clinical and skeletal muscle pathological characteristics of patients with anti Mi-2 antibody positive idiopathic inflammatory myopathy(IIM). Methods The clinical data of 7 anti Mi-2 antibody positive IIM patients diagnosed and treated in the First Medical Center of the PLA General Hospital from January 1, 2019 to February 28, 2022 were collected for retrospective analysis, and the literature at home and abroad was reviewed. Results In 7 cases, proximal muscle weakness was more common, which could be accompanied by dysphagia, myalgia, arthralgia and muscular atrophy. Severe rash occurred in 4 cases. Interstitial pneumonia(ILD) is rare, and may be accompanied by malignant tumor or hypothyroidism/vaccination history. The pathological changes of inflammatory myopathy can be seen in skeletal muscle pathology, which is consistent with the characteristics of perifascicular immunomyopathy(IMPP). Immunohistochemical staining showed positive expression of MHC-Ⅰ muscle underwear and positive expression of anti myxovirus A protein(MxA) perifascicular muscle fiber membrane and muscle plasma. After 3 months of combined treatment with glucocorticoid and immunosuppressant, the condition of 7 patients improved. Conclusion The clinical phenotypes of IIM patients with positive anti Mi-2 antibody were mostly dermatomyositis(DM) classic rash, myalgia, myasthenia, etc. IMPP can be seen in muscle biopsy, with less ILD, and may be associated with immune disease. Steroid combined with immunosuppressive agents had a better response.
作者
蔺颖
陈娟
刘孟洋
石强
Lin Ying;Chen Juan;Liu Mengyang;Shi Qiang(Department of Neurology,the First Medical Center,Chinese PLA General Hospital,Beijing 100853,China)
出处
《疑难病杂志》
CAS
2023年第1期78-81,89,共5页
Chinese Journal of Difficult and Complicated Cases
基金
国家自然科学基金面上项目(81771358)。
关键词
特发性炎性肌病
抗Mi-2抗体
肌活检
临床特征
Idiopathic inflammatory myopathy
Anti-Mi-2 antibody
Muscle biopsy
Clinical characteristic
