Internal Medicine, The University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA.
Pathology, Ballad Health, Johnson City, Tennessee, USA.
Heart. 2021 May;107(10):790-798. doi: 10.1136/heartjnl-2020-318041. Epub 2021 Jan 19.
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterised by multiorgan lymphoplasmacytic infiltration, obliterative phlebitis and storiform fibrosis. It can be associated with cardiovascular pathology. The objective of this narrative review is to summarise the published literature on cardiovascular manifestations of IgG4-RD and to provide a basis for diagnosis and management of the condition by the practising cardiologist.We propose the following categorisations of cardiovascular IgG4-RD: aortitis, medium-vessel arteritis, pulmonary vascular disease, phlebitis, valvulopathy, pericarditis, myocardial disease and antineutrophilic cytoplasmic antibody-associated vasculitis. We also review herein developments in radiological diagnosis and reported medical and surgical therapies. Cardiovascular lesions frequently require procedural and/or surgical interventions, such as aortic aneurysm repair and valve replacement. IgG4-RD of the cardiovascular system results in serious complications that can be missed if not evaluated aggressively. These are likely underdiagnosed, as clinical presentations frequently mimic cardiovascular disease due to more common aetiologies (myocardial infarction, abdominal aortic aneurysm and so on). While systemic corticosteroids are the mainstay of IgG4-RD treatment, biological and disease-modifying agents are becoming more widely used. Cardiologists should be aware of cardiovascular IgG4-RD as a differential diagnosis, and understand the roles of corticosteroids, disease-modifying agents and biologicals, as well as their integration with surgical approaches. There are several knowledge gaps, including diagnosis, risk factors, pathogenesis and appropriate management in Ig4-RD of the cardiovascular system. Areas lacking well-conducted randomized trials include safety of steroids in the setting of vascular aneurysms and the role of disease-modifying drugs and biological agents in patients with established cardiovascular complications of this multifaceted enigmatic disease.
免疫球蛋白 G4 相关疾病（IgG4-RD）是一种系统性纤维炎症性疾病，其特征为多器官淋巴浆细胞浸润、闭塞性静脉炎和席纹状纤维化。它可与心血管病理学相关。本叙述性综述的目的是总结 IgG4-RD 的心血管表现的已发表文献，并为临床心脏病学家的诊断和治疗提供依据。我们提出了以下心血管 IgG4-RD 的分类：大动脉炎、中血管动脉炎、肺血管疾病、静脉炎、瓣膜病、心包炎、心肌疾病和抗中性粒细胞胞质抗体相关性血管炎。我们还回顾了放射诊断的进展和报告的医学和手术治疗方法。心血管病变常需要进行程序和/或手术干预，例如主动脉瘤修复和瓣膜置换。如果不积极评估，心血管 IgG4-RD 会导致严重的并发症，这些并发症可能会被忽视。由于更常见的病因（心肌梗死、腹主动脉瘤等），这些疾病的临床表现常与心血管疾病相似，因此很可能被误诊。虽然全身性皮质类固醇是 IgG4-RD 治疗的主要方法，但生物制剂和疾病修饰剂的应用越来越广泛。心脏病学家应该意识到心血管 IgG4-RD 是一种鉴别诊断，并了解皮质类固醇、疾病修饰剂和生物制剂的作用，以及它们与手术方法的整合。还有几个知识空白，包括心血管 IgG4-RD 的诊断、危险因素、发病机制和适当的治疗方法。缺乏良好设计的随机试验的领域包括在血管动脉瘤中使用类固醇的安全性以及在这种多方面的神秘疾病已经存在心血管并发症的患者中使用疾病修饰药物和生物制剂的作用。
