Background Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by IgG4-positive plasma cell infiltration that can affect multiple organs, including the cardiovascular system. The diagnosis of IgG4-RD relies on a combination of clinical, serological, radiological and pathological findings. However, due to the varied and insidious clinical presentations, normal IgG4 levels in a significant percentage of patients and frequent multi-organ involvement, imaging plays a crucial role in the diagnosis of IgG4-RD. The aim of this study is to comprehensively examine the imaging findings in IgG4-related cardiovascular disease for accurate diagnosis and appropriate treatment. Methods A systematic search was conducted across the electronic databases PubMed, Scopus, Embase and Web of Science, to 1 September 2023, following PRISMA guidelines, searching for studies reporting detailed cardiovascular imaging findings in IgG4-RD. Results The search yielded 68 studies (60 case reports, 5 case series, 2 cross-sectional, 1 case–control) with 120 cases of cardiovascular IgG4-RD. Most of the cases were male, averaging 62.8 years. The common initial symptoms were dyspnoea and chest pain. The most common imaging finding was vasculopathy, including vessel wall thickening, periarteritits, periaortitis, aortitis, stenosis, ectasia, aneurysm formation, intramural haemorrhage, fistula formation and dissection, followed by pericardial involvement and mediastinal masses. Case series and cross-sectional studies also showed vasculopathy to be the most common finding on various imaging modalities, including angiography and PET/CT, highlighting the complex pathology of IgG4-RD. Conclusion This study evaluated current IgG4-RD articles, revealing a higher prevalence in men and vasculopathy as the most common cardiovascular complication.

中文翻译：

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IgG4 相关疾病心血管受累的影像学检查结果：一项系统评价研究


背景免疫球蛋白 G4 相关疾病 （IgG4-RD） 是一种纤维炎症性疾病，其特征是 IgG4 阳性浆细胞浸润，可影响多个器官，包括心血管系统。IgG4-RD 的诊断依赖于临床、血清学、放射学和病理学结果的组合。然而，由于 IgG4-RD 的临床表现多样且隐匿，很大一部分患者的 IgG4 水平正常且多器官受累频繁，影像学检查在 IgG4-RD 的诊断中起着至关重要的作用。本研究的目的是全面检查 IgG4 相关心血管疾病的影像学表现，以便准确诊断和适当治疗。方法 按照 PRISMA 指南，在 2023 年 9 月 1 日之前，在电子数据库 PubMed、Scopus、Embase 和 Web of Science 中进行了系统检索，检索了报告 IgG4-RD 中详细心血管影像学发现的研究。结果 检索得到 68 项研究 （60 例病例报告，5 例病例系列，2 例横断面，1 例对照），其中 120 例心血管 IgG4-RD。大多数病例为男性，平均年龄为 62.8 岁。常见的初始症状是呼吸困难和胸痛。最常见的影像学表现是血管病变，包括血管壁增厚、动脉周围炎、主动脉周围炎、主动脉炎、狭窄、扩张、动脉瘤形成、壁内出血、瘘管形成和夹层，其次是心包受累和纵隔肿块。病例系列和横断面研究还显示，血管病变是各种影像学检查方式（包括血管造影和 PET/CT）最常见的发现，突出了 IgG4-RD 的复杂病理。 结论 本研究评估了当前的 IgG4-RD 文章，揭示了男性患病率较高，血管病变是最常见的心血管并发症。