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ANCA相关血管炎和IgG4相关疾病之间的免疫细胞交叉:一个未解决的病原性联系

Cui Wang¹, Ronghua He², Xue Bai¹

¹Department of Nephrology and Rheumatology, The Second Hospital of Jilin University, Changchun, Jilin, China.

Frontiers in immunology

|November 3, 2025

概括

免疫球蛋白G4相关疾病 (IgG4-RD) 和抗中性粒细胞质抗体相关血管炎 (AAV) 可能会重叠,从而带来诊断挑战. 本综述探讨了这些罕见的多系统性疾病之间的潜在病理生理联系.

科学领域:

类风湿病学类风湿病学
免疫学免疫学免疫学
病理生理学病理生理学

背景情况:

免疫球蛋白G4相关疾病 (IgG4-RD) 是一种罕见的多系统性纤维炎症疾病,影响多个器官.
抗中性粒细胞质抗体相关性血管炎 (AAV) 包括EGPA,MPA和GPA,其特征是全身炎症.
已发现IgG4-RD和AAV之间的重叠综合征,由于共同的器官参与和血清学标记,这使得临床诊断复杂化.

研究的目的:

为了检查IgG4-RD和AAV (GPA/MPA) 重叠综合征之间的潜在病理生理联系.
为了阐明这些复杂的自身免疫性疾病的不清楚的潜在机制.

主要方法:

现有病例报告和IgG4-RD和AAV重叠研究的文献综述.
分析共享的免疫路径,包括细胞 (巨细胞,B细胞,T细胞) 和细胞因子的参与.
检查导致诊断困难的临床和血清学特征.

主要成果:

一些IgG4-RD测试阳性ANCA的患者,以及一些AAV患者显示IgG4水平升高或IgG4阳性血细胞透.
在重叠的情况下,建议共享涉及免疫细胞和炎症细胞因子的致病机制.
这些重叠条件的确切病理生理基础仍然不完全理解.

结论:

了解IgG4-RD和AAV之间的病理生理联系对于准确的诊断和管理至关重要.
需要进一步的研究来澄清驱动这些重叠的多系统性疾病的共享机制.
确定常见途径可能会导致IgG4-RD和AAV重叠的患者的新疗法策略.

关键词:

在AAV中,AAV是AAV,AAV是AAV.在GPA/MPA中使用.在IgG4-RD的研究中,覆盖综合征的重叠综合征病理生理学病理生理学

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