Sarcoidosis is a multi-systemic granulomatous disease of unknown origin. Recent research has focused upon the role of autoimmunity in its development and progression. This study aimed to determine and define the disturbance and distribution of T and B cell subsets in the alveolar and peripheral compartments. Thirteen patients were selected for the study [median age, interquartile range (IQR) = 57 years (48–59); 23% were male]. Twelve healthy controls [median age, IQR = 53 years (52–65); 16% male] were also enrolled into the study. Cellular and cytokine patterns were measured using the cytofluorimetric approach. Peripheral CD8 percentages were higher in sarcoidosis patients (SP) than healthy controls (HC) (p = 0.0293), while CD4 percentages were lower (p = 0.0305). SP showed low bronchoalveolar lavage (BAL) percentages of CD19 (p = 0.0004) and CD8 (p = 0.0035), while CD19⁺CD5⁺CD27⁻ percentages were higher (p = 0.0213); the same was found for CD4 (p = 0.0396), follicular regulatory T cells (T_reg) (p = 0.0078) and T_reg (p < 0.0001) cells. Low T helper type 17 (Th17) percentages were observed in BAL (p = 0.0063) of SP. Peripheral CD4⁺ C-X-C chemokine receptor (CXCR)5⁺CD45RA⁻) percentages and follicular T helper cells (Tfh)-like Th1 (Tfh1) percentages (p = 0.0493 and p = 0.0305, respectively) were higher in the SP than HC. Tfh1 percentages and Tfh-like Th2 percentages were lower in BAL than in peripheral blood (p = 0.0370 and p = 0.0078, respectively), while CD4⁺ C-X-C motif CXCR5⁺CD45RA⁻ percentages were higher (p = 0.0011). This is the first study, to our knowledge, to demonstrate a link between an imbalance in circulating and alveolar Tfh cells, especially CCR4-, CXCR3- and CXCR5-expressing Tfh subsets in the development of sarcoidosis. These findings raise questions about the pathogenesis of sarcoidosis and may provide new directions for future clinical studies and treatment strategies.

中文翻译：

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肺结节病的适应性免疫系统——外周和肺泡生物标志物的比较

结节病是一种起源不明的多系统肉芽肿性疾病。最近的研究集中在自身免疫在其发展和进展中的作用。本研究旨在确定和定义肺泡和外周隔室中 T 和 B 细胞亚群的干扰和分布。研究选择了 13 名患者 [中位年龄，四分位距 (IQR) = 57 岁 (48-59)；23% 是男性]。十二名健康对照 [中位年龄，IQR = 53 岁 (52–65)；16% 的男性] 也参加了这项研究。使用细胞荧光法测量细胞和细胞因子模式。结节病患者 (SP) 的外周 CD8 百分比高于健康对照 (HC) ( p  = 0.0293)，而 CD4 百分比较低 ( p = 0.0305）。SP 显示出低支气管肺泡灌洗 (BAL) 百分比的 CD19 ( p  = 0.0004) 和 CD8 ( p  = 0.0035)，而 CD19 ⁺ CD5 ⁺ CD27 ^-百分比较高 ( p  = 0.0213)；CD4 ( p  = 0.0396)、滤泡调节性 T 细胞 (T _reg ) ( p  = 0.0078) 和 T _reg ( p  < 0.0001) 细胞也是如此。在 SP 的BAL ( p = 0.0063)中观察到低 T 辅助类型 17 (Th17) 百分比。外周 CD4 ⁺ CXC 趋化因子受体 (CXCR)5 ⁺ CD45RA ^-) 百分比和滤泡 T 辅助细胞 (Tfh) 样 Th1 (Tfh1) 百分比（分别为p  = 0.0493 和p  = 0.0305）在 SP 中高于 HC。BAL 中的 Tfh1 百分比和 Tfh 样 Th2 百分比低于外周血（分别为p  = 0.0370 和p  = 0.0078），而 CD4 ⁺ CXC 基序 CXCR5 ⁺ CD45RA ^-百分比较高（p = 0.0011）。据我们所知，这是第一项证明循环和肺泡 Tfh 细胞失衡之间存在联系的研究，尤其是表达 CCR4、CXCR3 和 CXCR5 的 Tfh 亚群在结节病的发展过程中。这些发现对结节病的发病机制提出了质疑，并可能为未来的临床研究和治疗策略提供新的方向。