Large vessel vasculitis (LVV) is a type of vasculitis characterized by granulomatous inflammation of medium- and large-sized arteries. Clinical assessment of acute phase reactants has been conventionally used to diagnose and monitor diseases; however, accurate assessment of vascular disease activity status can be difficult. In this study, we investigated comprehensive immuno-phenotyping to explore useful biomarkers associated with clinical characteristics. Consecutive patients with newly diagnosed LVV who visited our institution between May 2016 and May 2019 were enrolled. The number of circulating T cells, B cells, natural killer cells, dendritic cells, monocytes, and granulocytes was examined and chronologically followed. Baseline and time-course changes in immuno-phenotyping associated with disease activity were assessed. Comprehensive immuno-phenotyping data from 90 samples from each of 20 patients with LVV were compared with those from healthy controls (HCs). The number of helper T (Th), follicular helper T (Tfh), CD8+ T, CD14++ CD16+ monocytes, and neutrophils were higher in patients with giant cell arteritis (GCA) and/or Takayasu arteritis (TAK) than in HCs. Among them, the number of CD8+ T and CD8+ Tem were higher in patients with TAK than in GCA. Notably, memory CD4+ and CD8+ T cells in patients with TAK remained high even in the remission phase. Further analysis revealed that the number of Th1, Th17, and Tfh cells was associated with disease relapse in GCA and TAK and that the number of CD8+ T cells was associated with relapse in TAK. Th1, Th17, and Tfh cells decreased after treatment with biologic agents, while CD8+ T cells did not. Our results from peripheral immuno-phenotyping analysis indicate that the numbers of Th and Tfh cells changed along with the disease condition in both GCA and TAK, while that of CD8+ T cells did not, especially in TAK. Treatment with biologic agents decreased the proportion of Th and Tfh cells, but not CD8+ T cells, in the patients. Chronological immuno-phenotyping data explained the difference in therapeutic response, such as reactivities against biologics, between GCA and TAK.

中文翻译：

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大血管血管炎的临床特征与周围免疫表型变化之间的显着关联

大血管血管炎（LVV）是一种血管炎，其特征是中型和大型动脉的肉芽肿性炎症。急性期反应物的临床评估通常用于诊断和监测疾病。但是，很难准确评估血管疾病的活动状态。在这项研究中，我们调查了全面的免疫表型，以探索与临床特征相关的有用的生物标志物。入选了2016年5月至2019年5月间来我院就诊的新诊断为LVV的连续患者。检查循环T细胞，B细胞，自然杀伤细胞，树突细胞，单核细胞和粒细胞的数量，并按时间顺序进行。评估了与疾病活动相关的免疫表型的基线和时程变化。将来自20例LVV患者每人的90个样品的综合免疫表型数据与健康对照（HCs）的数据进行了比较。巨细胞性动脉炎（GCA）和/或Takayasu动脉炎（TAK）患者的辅助性T（Th），滤泡性辅助性T（Tfh），CD8 + T，CD14 ++ CD16 +单核细胞和中性粒细胞的数量高于HCs。其中，TAK患者的CD8 + T和CD8 + Tem数量高于GCA。值得注意的是，即使在缓解期，TAK患者的记忆CD4 +和CD8 + T细胞仍保持较高水平。进一步的分析表明，Th1，Th17和Tfh细胞的数量与GCA和TAK中的疾病复发相关，而CD8 + T细胞的数量与TAK中的复发相关。用生物制剂处理后，Th1，Th17和Tfh细胞减少，而CD8 + T细胞则没有。我们的外周免疫表型分析结果表明，GCA和TAK中Th和Tfh细胞的数量随疾病状况而变化，而CD8 + T细胞的数量却没有变化，特别是在TAK中。用生物制剂治疗可降低患者的Th和Tfh细胞的比例，但不会降低CD8 + T细胞的比例。时序免疫表型数据解释了GCA和TAK之间治疗反应的差异，例如对生物制剂的反应性。