Tioni Chiara, Antonutti Marco, Di Nora Concetta, Proclemer Alessandro
Cardiology Division, Department of Cardiothoracic Science, Azienda Sanitaria Universitaria Integrata Santa Maria della Misericordia, Piazzale S. Maria della Misericorda 15, Udine, Italy.
Cardiovascular Department, Ospedali Riuniti, Via Valdoni, Trieste, Italy.
Eur Heart J Case Rep. 2018 Oct 16;2(4):yty111. doi: 10.1093/ehjcr/yty111. eCollection 2018 Dec.
Chest pain is a common reason for admission to the Emergency Department and aortic disease is a relatively frequent cause amongst the total number of admissions due to chest pain. IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated condition that mimics several malignant, infectious, and inflammatory disorders.
We report a rare case of IgG4-related aortitis complicated with severe aortic regurgitation and multivessel coronary artery disease in a 64-year-old man with a history of atypical chest pain. The diagnosis was made performing transthoracic echocardiography, transoesophageal echocardiography, and left heart catheterization; the aortitis was an incidental finding discovered by computed tomography angiography. Unusually, the positron emission tomography-computed tomography (PET-CT) scans did not reveal metabolic activity in the aortic wall. This last finding prompted us to exclude more aggressives arteritis (such as Horton's disease or Takayasu arteritis); syphilis infection and other infective or autoimmune diseases were excluded with laboratory tests. The patient underwent cardiac surgery with replacement of both the aortic valve and the ascending aorta, also performing a coronary aortic bypass graft (CABG). Despite the PET-CT scans were negative in the aortic wall, the histological specimens showed diffuse lymphoid infiltration, fibro-atheromatosis lesions, and medium-interstitial hyperplasia compatible with aortic atherosclerosis and IgG4-RD. The post-surgery course was free of complications and the patient was discharged in good clinical condition. He was referred to the Rheumatologic Department and a corticosteroid therapy has been started.
The growing recognition of IgG4-related systemic disease as a clinical entity underscores the importance of considering this diagnosis in patients with any type of idiopathic aortitis and aortic valve disease. Noteworthy, the PET-CT scans could be negative in this disease, so histological exams are mandatory to make the diagnosis; in our case an unusual post-surgery histopathological finding prompted us to diagnose a rare, unrecognized disease and allowed us to treat the patient properly.
胸痛是急诊科收治患者的常见原因，在因胸痛入院的患者总数中，主动脉疾病是相对常见的病因。IgG4相关性疾病（IgG4-RD）是一种多器官免疫介导的疾病，可模仿多种恶性、感染性和炎症性疾病。
我们报告了一例罕见的IgG4相关性主动脉炎病例，该病例发生在一名有非典型胸痛病史的64岁男性身上，并发严重主动脉瓣关闭不全和多支冠状动脉疾病。通过经胸超声心动图、经食管超声心动图和左心导管检查做出诊断；主动脉炎是计算机断层血管造影偶然发现的。不同寻常的是，正电子发射断层扫描-计算机断层扫描（PET-CT）未显示主动脉壁有代谢活性。这一发现促使我们排除更具侵袭性的动脉炎（如颞动脉炎或高安动脉炎）；通过实验室检查排除了梅毒感染及其他感染性或自身免疫性疾病。患者接受了心脏手术，置换了主动脉瓣和升主动脉，还进行了冠状动脉搭桥术（CABG）。尽管PET-CT扫描显示主动脉壁为阴性，但组织学标本显示弥漫性淋巴细胞浸润、纤维粥样病变以及与主动脉动脉粥样硬化和IgG4-RD相符的中度间质增生。术后过程无并发症，患者出院时临床状况良好。他被转诊至风湿科并开始了皮质类固醇治疗。
对IgG4相关性全身性疾病作为一种临床实体的认识不断增加，凸显了在任何类型的特发性主动脉炎和主动脉瓣疾病患者中考虑这一诊断的重要性。值得注意的是，这种疾病的PET-CT扫描可能为阴性，因此组织学检查对于做出诊断至关重要；在我们的病例中，术后不寻常的组织病理学发现促使我们诊断出一种罕见的、未被认识的疾病，并使我们能够对患者进行恰当治疗。
