Dendritic cells (DCs) accumulate in the lung of patients affected by idiopathic pulmonary fibrosis (IPF). We measured the frequencies of circulating conventional CD1c + and CD141+ cells (namely, cDC2 and cDC1) and of plasmacytoid CD303+ DCs in a cohort of 60 therapy naive IPF patients by flow cytometry. Peripheral levels of reactive oxygen species (ROS) and of pro-inflammatory and Th1/Th2 polarizing cytokines were also analyzed. All blood DC subtypes were significantly reduced in IPF patients in comparison to age- and sex-matched controls, while ROS and interleukin (IL-6) levels were augmented. IL-6 expression increased along with disease severity, according to the gender-age-physiology index, and correlated with the frequency of cDC2. IL-6 and cDC2 were not influenced by anti-fibrotic therapies but were associated with a reduced survival, the latter being an independent predictive biomarker of worse prognosis. Deciphering the role of DCs in IPF might provide information on disease pathogenesis and clinical behavior.

中文翻译：

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循环树突状细胞在特发性肺纤维化中严重减少，具有预测预后的潜在价值。

受特发性肺纤维化（IPF）影响的患者的肺中积累树突状细胞（DC）。我们通过流式细胞术测量了60名未接受过IPF治疗的初始患者中循环常规CD1c +和CD141 +细胞（即cDC2和cDC1）以及浆细胞CD303 + DC的频率。还分析了外周血中的活性氧（ROS）以及促炎和Th1 / Th2极化细胞因子的水平。与年龄和性别相匹配的对照组相比，IPF患者的所有血液DC亚型均显着降低，而ROS和白介素（IL-6）水平升高。根据性别-年龄-生理指标，IL-6表达随疾病的严重程度而增加，并与cDC2的发生频率相关。IL-6和cDC2不受抗纤维化疗法的影响，但与存活率降低相关，后者是预后较差的独立预测生物标志物。了解DC在IPF中的作用可能会提供有关疾病发病机理和临床行为的信息。