Abstract：
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a chronic, immune-mediated condition characterized by fibro-inflammatory lesions with lymphoplasmacytic infiltration. Diagnosis traditionally relies on histopathological findings, including the presence of IgG4+ plasma cells. However, due to challenges in biopsy accessibility, additional measures are needed to facilitate diagnosis.
OBJECTIVE: To identify additional parameters for characterizing IgG4-RD patients.
METHODS: We compared several circulating factors between a cohort of patients with IgG4-RD disease seen at our hospital between 2017 and 2023 and healthy controls.
RESULTS: Among 16 suspected patients, 13 were confirmed to have IgG4-RD, and 3 were classified as highly likely. Comparison with controls revealed differences in white blood cell count (WBC) (Folf change (FC) 1.46, P < 0.05), plasmablasts (FC 3.76, P< 0.05), plasmablasts CD38 (FC 1.43, P < 0.05), and CD27 (FC 0.66, P = 0.054), thus highlighting potential markers for IgG4-RD diagnosis. Treatments with steroids/rituximab tend to reduce plasmablast (FC 0.6) and IgG4 (FC 0.28) levels and to increase Gal-3 levels.
CONCLUSIONS: Levels of plasmablasts are a significant diagnostic feature in IgG4-RD. Healthy individuals have a lower level of plasmablasts. Elevated Gal-3 in serum of patients with IgG4-RD suggests a role in plasmablast activation. CD38/CD27 expression by plasmablasts emerges as a potential marker. Further research on a larger cohort is needed to confirm these findings.
OBJECTIVE: To identify additional parameters for characterizing IgG4-RD patients.
METHODS: We compared several circulating factors between a cohort of patients with IgG4-RD disease seen at our hospital between 2017 and 2023 and healthy controls.
RESULTS: Among 16 suspected patients, 13 were confirmed to have IgG4-RD, and 3 were classified as highly likely. Comparison with controls revealed differences in white blood cell count (WBC) (Folf change (FC) 1.46, P < 0.05), plasmablasts (FC 3.76, P< 0.05), plasmablasts CD38 (FC 1.43, P < 0.05), and CD27 (FC 0.66, P = 0.054), thus highlighting potential markers for IgG4-RD diagnosis. Treatments with steroids/rituximab tend to reduce plasmablast (FC 0.6) and IgG4 (FC 0.28) levels and to increase Gal-3 levels.
CONCLUSIONS: Levels of plasmablasts are a significant diagnostic feature in IgG4-RD. Healthy individuals have a lower level of plasmablasts. Elevated Gal-3 in serum of patients with IgG4-RD suggests a role in plasmablast activation. CD38/CD27 expression by plasmablasts emerges as a potential marker. Further research on a larger cohort is needed to confirm these findings.
摘要：
背景：免疫球蛋白G4相关疾病（IgG4-RD）是一种慢性疾病，免疫介导的疾病，其特征是具有淋巴浆细胞浸润的纤维炎性病变。传统上，诊断依赖于组织病理学发现，包括IgG4+浆细胞的存在。然而,由于活检可及性方面的挑战，需要采取其他措施来促进诊断.
目的：确定用于表征IgG4-RD患者的其他参数。
方法：我们比较了2017年至2023年在我们医院就诊的IgG4-RD患者队列与健康对照组之间的几个循环因素。
结果：在16名疑似患者中，13人被证实患有IgG4-RD，和3被列为极可能。与对照组比较显示白细胞计数（WBC）（Folf变化（FC）1.46，P<0.05），成浆细胞（FC3.76，P<0.05），浆细胞CD38（FC1.43，P<0.05），和CD27(FC0.66，P=0.054)，因此突出了IgG4-RD诊断的潜在标志物。使用类固醇/利妥昔单抗的治疗倾向于降低血浆母细胞(FC0.6)和IgG4(FC0.28)水平并增加Gal-3水平。
结论：成浆细胞水平是IgG4-RD的重要诊断特征。健康个体具有较低水平的成浆细胞。IgG4-RD患者血清中Gal-3的升高提示在浆细胞活化中起作用。成浆细胞的CD38/CD27表达成为潜在的标志物。需要对更大的队列进行进一步的研究来证实这些发现。
目的：确定用于表征IgG4-RD患者的其他参数。
方法：我们比较了2017年至2023年在我们医院就诊的IgG4-RD患者队列与健康对照组之间的几个循环因素。
结果：在16名疑似患者中，13人被证实患有IgG4-RD，和3被列为极可能。与对照组比较显示白细胞计数（WBC）（Folf变化（FC）1.46，P<0.05），成浆细胞（FC3.76，P<0.05），浆细胞CD38（FC1.43，P<0.05），和CD27(FC0.66，P=0.054)，因此突出了IgG4-RD诊断的潜在标志物。使用类固醇/利妥昔单抗的治疗倾向于降低血浆母细胞(FC0.6)和IgG4(FC0.28)水平并增加Gal-3水平。
结论：成浆细胞水平是IgG4-RD的重要诊断特征。健康个体具有较低水平的成浆细胞。IgG4-RD患者血清中Gal-3的升高提示在浆细胞活化中起作用。成浆细胞的CD38/CD27表达成为潜在的标志物。需要对更大的队列进行进一步的研究来证实这些发现。
