Wang Yan, Tian Feng, Li Hui
Department of Gastroenterology, Shengjing Hospital of China Medical University, Shenyang, Liaoning, China.
Front Immunol. 2025 Jul 4;16:1625456. doi: 10.3389/fimmu.2025.1625456. eCollection 2025.
IgG4-related disease (IgG4-RD) is a chronic fibrotic inflammatory condition characterized by elevated serum IgG4 levels and the infiltration of IgG4-bearing plasma cells in affected organs. It can involve various organs, particularly large vessels. IgG4-related aortitis/periaortitis and periarteritis (IgG4-related PAO/PA) predominantly affect the abdominal aorta and iliac arteries, with a higher prevalence in elderly males. This condition exhibits distinct clinical, histologic, and radiological features compared to IgG4-RD without vascular involvement and other immune-associated vasculitides. IgG4-related PAO/PA diagnosis primarily relies on histopathological findings and imaging studies. Glucocorticoids (GCs) are the mainstay of treatment, often combined with immunosuppressants (IMs), while B- and T-cell-targeted therapies are under investigation. Although most patients respond well to treatment, the disease can be life-threatening due to complications such as myocardial infarction, aortic dissection, and aneurysmal rupture. Therefore, understanding these characteristics is crucial for clinicians to make accurate diagnoses and implement effective treatment strategies. This review provides a comprehensive overview of the current understanding of the pathogenesis, histopathological characteristics, clinical features, diagnosis, treatment, and prognosis of IgG4-related PAO/PA.
IgG4相关性疾病（IgG4-RD）是一种慢性纤维化炎症性疾病，其特征为血清IgG4水平升高以及受累器官中存在携带IgG4的浆细胞浸润。它可累及多个器官，尤其是大血管。IgG4相关性主动脉炎/主动脉周炎和动脉周炎（IgG4相关性PAO/PA）主要累及腹主动脉和髂动脉，在老年男性中患病率较高。与无血管受累的IgG4-RD及其他免疫相关血管炎相比，这种疾病具有独特的临床、组织学和放射学特征。IgG4相关性PAO/PA的诊断主要依靠组织病理学检查结果和影像学研究。糖皮质激素（GCs）是主要治疗药物，常与免疫抑制剂（IMs）联合使用，而针对B细胞和T细胞的疗法正在研究中。尽管大多数患者对治疗反应良好，但由于心肌梗死、主动脉夹层和动脉瘤破裂等并发症，该疾病可能危及生命。因此，了解这些特征对于临床医生做出准确诊断和实施有效的治疗策略至关重要。本综述全面概述了目前对IgG4相关性PAO/PA的发病机制、组织病理学特征、临床特征、诊断、治疗及预后的认识。
