Liu Xiaoyan, Zhao Yan, Wu Naqiong, Zhang Wenjia
Department of Cardiology, Fuwai Hospital, National Center for Cardiovascular Diseases, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishi Road, Xicheng District, Beijing China.
Eur Heart J Case Rep. 2022 Apr 26;6(5):ytac182. doi: 10.1093/ehjcr/ytac182. eCollection 2022 May.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition that affects multiple organs and systems.
A 51-year-old man with a history of occult left apex myocardial infarction diagnosed based on electrocardiographic and echocardiographic findings underwent coronary computed tomography (CT) angiography for the evaluation of coronary artery disease; the findings revealed a soft-tissue mass that surrounded the aortic root and the distal portion of the left coronary artery. The mass was considered an inflammatory lesion; high glucose uptake on positron emission tomography/CT supported this assumption. Coronary angiography revealed 80% stenosis of the distal portion of the left anterior descending artery, which corresponded with the infarction. Intravascular ultrasound revealed hypoechoic regions outside the lumina of the stenotic segment. Based on these findings, IgG4-related periaortitis/periarteritis was suspected; the patient was accordingly treated with oral prednisone and methotrexate. At the 3-month follow-up, the periaortic mass had slightly reduced in size.
Identification and diagnosis of IgG4-related cardiovascular disease are challenging; cases with localized coronary artery involvement may be misdiagnosed as atherosclerotic coronary artery disease. Although imaging techniques, including intracoronary imaging, may aid in differential diagnosis, their sensitivity and specificity still warrant further studies. Practical criteria that facilitate diagnosis and a better understanding of the disease are required.
免疫球蛋白G4相关性疾病(IgG4-RD)是一种影响多个器官和系统的免疫介导性疾病。
一名51岁男性,根据心电图和超声心动图检查结果诊断为隐匿性左心尖心肌梗死,因评估冠状动脉疾病接受了冠状动脉计算机断层扫描(CT)血管造影;检查结果显示主动脉根部和左冠状动脉远端周围有一个软组织肿块。该肿块被认为是炎性病变;正电子发射断层扫描/CT上的高葡萄糖摄取支持了这一假设。冠状动脉造影显示左前降支远端80%狭窄,与梗死部位相符。血管内超声显示狭窄节段管腔外的低回声区域。基于这些发现,怀疑为IgG4相关性主动脉周炎/动脉周炎;因此,该患者接受了口服泼尼松和甲氨蝶呤治疗。在3个月的随访中,主动脉周围肿块大小略有缩小。
IgG4相关性心血管疾病的识别和诊断具有挑战性;局限性冠状动脉受累的病例可能被误诊为动脉粥样硬化性冠状动脉疾病。尽管包括冠状动脉内成像在内的成像技术可能有助于鉴别诊断,但其敏感性和特异性仍有待进一步研究。需要有助于诊断和更好理解该疾病的实用标准。