以心肌炎为突出表现的特发性炎性肌病5例分析

Analyses of five cases of idiopathic inflammatory myopathies characterized by myocarditis

目的报道一组以心肌炎为突出表现的特发性炎性肌病(IIMs)患者的临床与病理特点。方法回顾性分析2017年1月至2021年11月北京大学第一医院就诊的5例IIMs合并心肌炎患者的临床、血清学以及病理学资料。结果5例患者中,男3例、女2例,发病年龄47~70岁,中位数为58岁,发病至确诊的时间为2~49个月,中位时间为21个月。3例患者慢性起病,以活动性喘憋、胸闷为突出表现,伴轻度骨骼肌力弱,血清抗线粒体抗体(AMA)阳性,心脏成像检查显示扩张性心肌病;1例患者亚急性起病,以力弱、呼吸困难为主要表现,血清抗信号识别颗粒(SRP)抗体弱阳性;1例患者急性起病,表现为晕厥、胸闷,四肢肌力正常,自身抗体检测阴性,肌电图检查显示右胫前肌呈肌源性损害。5例患者行骨骼肌活检,均呈炎性肌病病理改变。5例患者均接受免疫治疗,除1例在短暂好转后出现恶性心律失常而猝死外,其余4例症状均明显改善。结论部分IIMs患者以心肌炎为突出表现,伴或不伴四肢力弱,AMA阳性多见,外周组织骨骼肌病理检查有助于明确诊断。

Objective To report the clinical and pathological features of idiopathic inflammatory myopathies(IIMs)characterized by prominent cardiac involvement.Methods Retrospective analyses of clinical,serological,imaging and pathological data of five patients diagnosed as IIMs with myocarditis in Peking University First Hospital from January 2017 to November 2021 were performed.Results We included three men and two women,with an onset age range of 47-70 years and a median onset age of 58 years.The range of disease duration from onset to the diagnosis was 2-49 months,with a median of 21 months.Three patients had chronic onsets,with prominent manifestations of exertional dyspnea,chest discomfort,and mild muscle weakness,accompanied by positivity of anti-mitochondrial antibodies(AMA).Echocardiography and myocardial MRI suggested dilated cardiomyopathy.One patient presented with subacute onset of involvements of striated muscle,including limb weakness,dysarthria and dyspnea,accompanied by weak positivity of antibody to signal recognition particle(SRP).One patient presented with acute onset of syncope and chest tightness,with normal muscle strength and negativity of autoantibody,while whose electromyography showed a myogenic lesion of right tibialis anterior muscle.Skeletal muscle biopsies of all the five patients showed changes of inflammatory myopathies.All patients received immunotherapy,which showed effective in four patients,while the other patient suffered sudden death due to malignant arrhythmia six months after discharge.Conclusions In this report,patients with IIMs were characterized by prominent myocarditis,with or without skeletal muscle weakness.AMA positive is common in these patients.Skeletal muscle biopsy can provide valuable evidence for the diagnosis.