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病例报告
皮肌炎致心肌炎一例的临床特征及CMR影像表现
张艳翎 张燕 汪春红 朱秀梅 邹君鑫 黄茜

Cite this article as: Zhang YL, Zhang Y, Wang CH, et al. Clinical features and CMR imaging findings of myocarditis induced by dermatomyositis: One case report[J]. Chin J Magn Reson Imaging, 2022, 13(8): 96-97.本文引用格式:张艳翎, 张燕, 汪春红, 等. 皮肌炎致心肌炎一例的临床特征及CMR影像表现[J]. 磁共振成像, 2022, 13(8): 96-97. DOI:10.12015/issn.1674-8034.2022.08.020.


[关键词] 皮肌炎;特发性炎症性肌病;心肌炎;心脏磁共振
[Keywords] dermatomyositis;idiopathic inflammatory myopathies;myocarditis;cardiac magnetic resonance

张艳翎    张燕 *   汪春红    朱秀梅    邹君鑫    黄茜   

贵州医科大学附属医院放射科,贵阳 550000

张燕,E-mail:102651873@qq.com

作者利益冲突声明:全体作者均声明无利益冲突。


收稿日期:2021-09-14
接受日期:2022-07-28
中图分类号:R445.2  R593.26  R542.21 
文献标识码:B
DOI: 10.12015/issn.1674-8034.2022.08.020
本文引用格式:张艳翎, 张燕, 汪春红, 等. 皮肌炎致心肌炎一例的临床特征及CMR影像表现[J]. 磁共振成像, 2022, 13(8): 96-97. DOI:10.12015/issn.1674-8034.2022.08.020

       本文为回顾性研究,经过贵州医科大学附属医院伦理委员会批准,免除受试者知情同意,批准日期:2022年7月5日。

       患者女,32岁,以“全身皮疹4个月余,四肢肌肉无力1个月余”于2020年9月3日收入我院。专科检查示:全身可见紫红色斑片,头面部、胸部、背部、四肢可见暗红色斑片,伴脱屑。双下肢压痛、肌力减低。肌电图、神经传导检查提示:肌源性损害(广泛)。2020年9月10日取左上肢肱二头肌肌肉活检示:皮肌炎(dermatomyositis, DM)。给予激素大剂量冲击治疗并加用人免疫球蛋白冲击治疗,患者皮疹及肌肉压痛、无力较前好转。患者自诉病程中出现咳嗽、咳白色泡沫痰,活动后稍感胸闷、气促,时有盗汗、心悸,且住院治疗期间仍有胸闷、活动后气促症状。2020年9月7日行心脏超声提示心内形态及血流未见明显异常。2020年9月14日行心电图(electrocardiogram, ECG)提示:窦性心动过速,心率116 bpm。2020年9月16日及21日完善实验室检查。(1)心肌酶及标志物:肌酸激酶281.17 U/L,肌酸激酶同工酶33.30 U/L,N-末端脑钠肽前体(NT-proBNP)1316.00 pg/mL,肌钙蛋白T 0.0530 ng/mL,肌红蛋白153.60 ng/mL;(2)血沉:39 mm/h;(3)血常规:血红蛋白105.00 g/L,红细胞比容32.80%;(4)白介素-6 18.17 pg/mL;(5)D-二聚体2.47 ug/mL;(6)抗环瓜氨酸肽抗体5.243%。患者入院期间心肌标志物持续异常,为进一步明确有无心脏损害,行心脏形态学平扫、增强检查及心功能分析。(1)左心室心功能分析:射血分数(ejection fraction, EF)值38.9%,心输出量4.55 L/min,收缩末期容积(end-systolic volume)67.81 mL,舒张末期容积(end-diastolic volume)111.11 mL,舒张末期—收缩末期心脏质量(ED-ES mass)67.89 g;(2)平扫及增强:左心稍大,左室收缩及舒张运动幅度稍减低,T2WI提示信号较骨骼肌稍高,延迟强化(late gadalinum enhancement, LGE)左室前室间隔及游离壁心肌心外膜下见片状强化;(3)心肌灌注:左室整体初始T1-mapping(native T1-mapping)值(1320 ms)、细胞外容积(extracellular volume, ECV)值(32.7%)均大于正常高限。心脏磁共振(cardiac magnetic resonance, CMR)诊断:可符合DM所致心肌炎改变。后予以β受体阻滞剂等治疗改善心功能,随后患者好转出院,并遵医嘱于2020年11月30日门诊复查心肌酶及标志物、超声心动图:肌酸激酶(44.00 U/L)及肌酸激酶同工酶(7.50 U/L)未提示增高,肌钙蛋白T(0.0160 ng/mL)较前下降。超声心动图提示二尖瓣轻度返流。患者因个人经济原因未行CMR复查。

图1  女,32岁,皮肌炎致心肌炎。1A:收缩末期四腔心电影序列,可见左心室心腔缩小(箭);1B:舒张末期四腔心电影序列,可见左心室心腔扩大(箭),收缩末期(1A)与舒张末期(1B)相比,左心室心腔缩小动态改变不明显,提示心脏舒张功能减低;1C:左室短轴位T2快速自旋回波(FSE)序列,心肌信号稍高于邻近骨骼肌(箭);1D:左室短轴位延迟强化,左室前室间隔及游离壁心外膜下片状强化(箭);1E:整体左室整体初始T1-mapping值(1320 ms)增高;1F:左室整体细胞外容积(ECV)值(32.7%)大于正常高限。
Fig. 1  Female, 32 years old, myocarditis induced by dermatomyositis. 1A: The end-systole at the four chamber cine sequence, left ventricular ventricle was reduced (arrow); 1B: The end-diastole at the four chamber cine sequence, left ventricular cavity was dilated (arrow), the dynamic change of left ventricular heart cavity shrinkage was not obvious compared with the diastolic stage, and the diastolic function is reduced; 1C: Short axial T2 fast spin echo (FSE) sequence of the left ventricle; myocardial signal was slightly higher than that of adjacent skeletal muscle; 1D: Short axial late gadalinum enhancement (LGE) of the left ventricle, LGE with strip pattern was localized at the subepicardial layer of interventricular septum and free wall (arrow); 1E: Native T1-mapping value (1320 ms) was greater than the normal high limit; 1F: Extracellular volume value (32.7%) was greater than the normal high limit.

讨论

       DM为自身免疫性疾病,女性发病率较男性高,以皮肤及肌肉受累为主[1],关于DM患者心脏受累的报道并不多见。近年的研究发现6%~75%的DM患者存在心脏受累[2],心脏受累是其预后不良的主要因素之一,但炎症性肌病心脏损伤临床表现隐匿,患者可无症状或仅ECG提示心律不齐。因此,即使是无症状患者,尽早进行心肌损伤的检测对患者预后尤为重要。

       本例患者为年轻女性,首先以皮肤及肌肉症状来诊,随后出现活动后胸闷、气促,入院后实验室检查提示肌钙蛋白和NT-proBNP持续升高,提示存在心脏损害。而行ECG及超声心动图均未提示异常。结合本例患者CMR影像表现及既往文献[3, 4, 5]报道,该病例影像表现为:(1)左室EF值明显减低,收缩及舒张功能减低,以舒张功能减低为主;(2)T2WI信号较骨骼肌增高;(3)LGE可见左室前室间隔及游离壁心外膜下心肌异常强化;(4)左室整体native T1-mapping、ECV值增高。

       实验室检查、ECG及超声心动图是临床常用的筛查DM患者心脏情况的方法,但这些检查方法的敏感性和特异性常有差异,对评估心肌损伤及损伤程度的敏感性不足[6, 7]。而CMR能“一站式”对心脏形态、运动及心肌活性情况进行评价,是评估左室结构和功能参数的金标准[8],CMR组织特征成像技术(包括LGE、native T1-mapping、T2及ECV)能在活体内检测心肌损伤,尤其LGE技术作为评估体内心肌瘢痕的“金标准”[9],能在早期敏感地发现心肌损伤和/及纤维化,CMR mapping技术在不同疾病的动态监测中具有良好的可重复性和敏感性,能发现尚未引起显著左心室功能障碍的心肌损伤。本例患者行CMR后提示患者左室EF值明显减低,舒张功能减低,这与Feng等[3]、刘颖娴等[4]的研究一致,即在不同类型的炎性肌病患者可有不同程度的EF降低,这与炎性肌病类型有关,且约60%患者合并舒张功能下降。尽管目前对DM患者心肌受累的细胞和分子病理生理机制尚不明确,但T2WI高于正常基线提示了心肌炎症可能是DM患者心肌损伤的主要病理生理改变[10]。本例患者左室心肌T2WI信号稍增高,LEG提示左室前室间隔及游离壁心肌心外膜下片状强化的表现,也符合DM导致心肌炎的强化改变[3],这一LGE模式也可与其他自身免疫性疾病导致心肌炎相鉴别,如系统性红斑狼疮心肌炎常表现为左室后外侧壁心外膜至心肌中层散在强化,结节病心肌炎表现为左室心肌全层多形性强化,关节炎心肌炎表现为左室后外侧壁心肌中层线样强化[11]。本例患者左室总体native T1-mapping值、ECV值大于正常高限,也与文献一致[3, 4,12, 13, 14, 15]。Feng等[3]的研究发现,即使在左室心肌LGE阴性的DM患者,整体native T1-mapping值、ECV值也较正常人群增高,这也提示了在弥漫性心肌损伤的患者中,native T1-mapping值、ECV值的敏感性较LGE高。

       DM致心肌炎患者临床表现常缺乏特异性,病程早期心肌多因炎性水肿而对免疫抑制治疗敏感。CMR左心功能分析及LGE、native T1-mapping值、ECV值等组织特征成像技术对心肌活性评价的敏感性高,有助于在早期发现心肌损害,对改善患者预后有重要意义。

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