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原发性纤毛运动障碍患者的单核细胞表现出增强的炎症特性，并产生更高水平的促炎细胞因子。

Monocytes from patients with Primary Ciliary Dyskinesia show enhanced inflammatory properties and produce higher levels of pro-inflammatory cytokines.

机构信息

Department of Microbiology and Immunology, Rega Institute for Medical Research, Laboratory of Molecular Immunology, University of Leuven, Leuven, Belgium.

Unité de Pneumologie Pédiatrique et Mucoviscidose, Clinique Universitaire Saint-Luc UCL Bruxelles, Bruxelles, Belgium.

出版信息

Sci Rep. 2017 Nov 7;7(1):14657. doi: 10.1038/s41598-017-15027-y.

DOI:10.1038/s41598-017-15027-y
PMID:29116124
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5676706/
Abstract

Patients with Primary Ciliary Dyskinesia (PCD) suffer from recurrent upper and lower airway infections due to defects in the cilia present on the respiratory epithelium. Since chronic inflammatory conditions can cause changes in innate immune responses, we investigated whether monocytes isolated from the peripheral blood of pediatric PCD patients respond differently to inflammatory stimuli, compared to monocytes from healthy children and adults. The receptor for C5a (C5aR) was upregulated in PCD, whereas expression levels of the leukocyte chemoattractant receptors CCR1, CCR2, CCR5, BLT1 and FPR1 on PCD monocytes were similar to those on monocytes from healthy individuals. Also in vitro migration of PCD monocytes towards the ligands of those receptors (CCL2, fMLP, C5a and LTB4) was normal. Compared to healthy children, PCD patients had a higher percentage of the non-classic monocyte subset (CD14+CD16++) in circulation. Finally, PCD monocytes produced higher levels of pro-inflammatory cytokines (IL-1β and TNF-α) and chemokines (CCL3, CCL5, CCL18 and CCL22) in response to LPS, peptidoglycan and/or dsRNA stimulation. These data suggest that monocytes might exacerbate inflammatory reactions in PCD patients and might maintain a positive feedback-loop feeding the inflammatory process.

摘要

原发性纤毛运动障碍（PCD）患者由于呼吸道上皮纤毛存在缺陷而反复发生上呼吸道和下呼吸道感染。由于慢性炎症状态可导致固有免疫反应发生变化，我们研究了来自儿科 PCD 患者的外周血单核细胞与健康儿童和成人的单核细胞相比，对炎症刺激的反应是否不同。PCD 中 C5a 受体（C5aR）上调，而 PCD 单核细胞上的白细胞趋化因子受体 CCR1、CCR2、CCR5、BLT1 和 FPR1 的表达水平与健康个体的单核细胞相似。此外，PCD 单核细胞向这些受体的配体（CCL2、fMLP、C5a 和 LTB4）的体外迁移也是正常的。与健康儿童相比，PCD 患者循环中的非经典单核细胞亚群（CD14+CD16++）比例更高。最后，PCD 单核细胞在 LPS、肽聚糖和/或 dsRNA 刺激下产生更高水平的促炎细胞因子（IL-1β和 TNF-α）和趋化因子（CCL3、CCL5、CCL18 和 CCL22）。这些数据表明，单核细胞可能会加重 PCD 患者的炎症反应，并可能维持促进炎症过程的正反馈循环。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1bc7/5676706/cffdd587b6af/41598_2017_15027_Fig7_HTML.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1bc7/5676706/46f81ae275a3/41598_2017_15027_Fig1_HTML.jpg
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