Background: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immunological disorder characterized by fibro-inflammatory conditions; however, the pathobiology of IgG4-RD has not been fully identified. Objective: This study aimed to analyze systemic differences of innate and adaptive immune cells from healthy controls and patients with IgG4-RD. Methods: Healthy controls (n = 9) and IgG4-RD patients (n = 7) were recruited with informed consent. Peripheral blood was collected from healthy controls and IgG4-RD patients, and three blood samples from IgG4-RD patients were re-collected two months after the last rituximab (RTX) treatment. The various immune cells and cytokine productions were measured by flow cytometry. Results: Blood CD14⁺ monocytes and steady-state follicular helper T cells were increased in patients with IgG4-RD. However, there were no changes in other immune cell populations, including B cells, CD4 T cells, CD8 T cells, and innate lymphoid cells. Also, the TGF-β-producing CD14⁺ monocytes were significantly augmented in patients with IgG4-RD. Two months after RTX treatment, total B cells (CD19⁺) were depleted; however, the expressions of TGF-β from CD14⁺ monocytes remained unchanged. Conclusion: These findings showed that IgG4-RD is related to the increment of CD14⁺ monocytes. Besides, controlling increased TGF-β-producing CD14⁺ monocytes with RTX treatment might be a conducive way to regulate IgG4-RD.
Int Arch Allergy Immunol

中文翻译：

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IgG4相关疾病患者的先天性和适应性免疫学特征分析。

背景：免疫球蛋白G4相关疾病（IgG4-RD）是一种以纤维炎性疾病为特征的全身性免疫疾病。然而，IgG4-RD的病理生物学尚未完全确定。目的：本研究旨在分析健康对照和IgG4-RD患者的先天和适应性免疫细胞的系统差异。方法：在知情同意下募集健康对照（ n = 9）和IgG4-RD患者（ n = 7）。在最后一次利妥昔单抗（RTX）治疗后两个月，从健康对照和IgG4-RD患者中收集外周血，并从IgG4-RD患者中收集三份血液样品。通过流式细胞术测量各种免疫细胞和细胞因子的产生。结果： IgG4-RD患者的血液CD14 ⁺单核细胞和稳态滤泡辅助性T细胞增加。但是，其他免疫细胞群没有变化，包括B细胞，CD4 T细胞，CD8 T细胞和先天性淋巴样细胞。同样，IgG4-RD患者中产生TGF-β的CD14 ⁺单核细胞显着增加。RTX治疗后两个月，总B细胞（CD19 ⁺）耗尽；然而，CD14 ⁺单核细胞中TGF-β的表达保持不变。结论：这些发现表明IgG4-RD与CD14 ⁺单核细胞的增加有关。此外，控制增加产生TGF-β的CD14 ⁺RTX处理的​​单核细胞可能是调节IgG4-RD的有益方法。
Int Arch过敏免疫