Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan.
Division of Rheumatology, Department of Internal Medicine, Fujita Health University School of Medicine, Aichi, Japan.
Rheumatology (Oxford). 2021 Feb 1;60(2):967-975. doi: 10.1093/rheumatology/keaa648.
To clarify relevant proteins and clinical characteristics of a phenotype of IgG4-related disease (IgG4-RD) with lymphadenopathy.
We enrolled patients newly diagnosed with IgG4-RD in our department between January 2000 and June 2018 and performed proteomic analysis to measure serum concentrations of 1305 proteins. We extracted proteins overexpressed in patients with IgG4-RD with lymphadenopathy by comparing between those with lymphadenopathy, those without lymphadenopathy and healthy controls. We further reviewed all the patients with IgG4-RD in our institution and investigated the characteristics and prognosis of the patients with IgG4-RD with lymphadenopathy.
Eighty-five patients with IgG4-RD were enrolled, of which, 55% had lymphadenopathy. Proteomic analysis in 31 patients with IgG4-RD and 6 healthy controls revealed that eotaxin-3 was a potential serum biomarker in the patients with lymphadenopathy versus those without lymphadenopathy and healthy controls. A cohort of 85 patients with IgG4-RD demonstrated that patients with lymphadenopathy showed a significantly higher serum IgG4, IgG4:IgG ratio, IgG4-RD responder index and eosinophilia (P < 0.001 for all), irrelevant of the extent to which organ involvement developed. Patients with lymphadenopathy treated with glucocorticoid alone relapsed with significantly higher rates than those without lymphadenopathy (P = 0.03).
Lymphadenopathy in IgG4-RD represents a phenotype associated with high disease activities, eosinophilia and relapsing disease. Eotaxin-3 is a novel biomarker related to IgG4-RD with lymphadenopathy.
阐明伴有淋巴结病的 IgG4 相关疾病（IgG4-RD）表型的相关蛋白和临床特征。
我们招募了 2000 年 1 月至 2018 年 6 月期间在我们科室新诊断为 IgG4-RD 的患者，并进行了蛋白质组学分析以测量 1305 种蛋白质的血清浓度。我们通过比较伴有淋巴结病、不伴有淋巴结病和健康对照组患者之间的差异，提取出 IgG4-RD 伴有淋巴结病患者中过度表达的蛋白。我们进一步回顾了我们机构的所有 IgG4-RD 患者，并调查了伴有淋巴结病的 IgG4-RD 患者的特征和预后。
共纳入了 85 例 IgG4-RD 患者，其中 55%伴有淋巴结病。对 31 例 IgG4-RD 患者和 6 例健康对照者的蛋白质组学分析显示，嗜酸性粒细胞趋化因子 3（eotaxin-3）是伴有淋巴结病患者与不伴有淋巴结病和健康对照组患者血清生物标志物。在 85 例 IgG4-RD 患者队列中，伴有淋巴结病的患者血清 IgG4、IgG4:IgG 比值、IgG4-RD 应答指数和嗜酸性粒细胞计数明显更高（所有 P 值均<0.001），而与器官受累程度无关。单独使用糖皮质激素治疗的伴有淋巴结病患者复发率明显高于无淋巴结病患者（P=0.03）。
IgG4-RD 中的淋巴结病代表了一种与高疾病活动度、嗜酸性粒细胞增多和复发性疾病相关的表型。嗜酸性粒细胞趋化因子 3 是一种与伴有淋巴结病的 IgG4-RD 相关的新型生物标志物。
