New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa

New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa
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大疱性表皮松解症中瘙痒介质和促炎细胞因子的新见解

DOI:
10.1002/cia2.12230
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发表时间:
2022
影响因子:
1
通讯作者:
Abe Riichiro
Abe Riichiro
中科院分区:
--
文献类型:
--
作者:
Nguyen Hong Ha;Shinkuma Satoru;Hayashi Ryota;Katsumi Tatsuya;Nishiguchi Tomoki;Natsuga Ken;Fujita Yasuyuki;Abe Riichiro

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目的大疱性表皮松解症(EB)是一种遗传性疾病,其特征为机械应力诱导的起泡。在严重EB亚型中观察到的皮外并发症如心肌病和肾脏疾病的存在,以及瘙痒是所有EB亚型的共同症状这一事实表明,EB不仅是一种皮肤脆弱性疾病,也是一种全身性炎症性疾病。我们的研究旨在阐明EB患者全身性炎症的基础。方法采用Luminex细胞因子法和酶联免疫吸附法对20例EB患者的血清进行分析。结果EB患者血清sIL‐2R、IL‐6、HGF、M‐CSF、SCGF‐β、IL‐8、IL‐16、IFN‐γ、MIF、MIP‐1α、胸腺基质淋巴生成素(TSLP)水平(分别为p< 0.01、p< 0.01、p< 0.01、p< 0.01、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.05、p< 0.01)显著升高,TNF‐β水平(p< 0.01)显著降低。包括IL - 4、IL - 5和IL - 13在内的Th2细胞因子在EB患者中未升高。相比之下,患者的TSLP显著升高,IL - 31和肿瘤抑制素M无统计学意义,但倾向于高于健康对照组。在促炎细胞因子如IL - 1β、IL - 6和TNF - α中,IL - 6在EB患者中升高。结论多种瘙痒介质和促炎细胞因子存在失衡。针对患者血清中发现的细胞因子升高的生物制剂被认为是EB的有益治疗选择。
ObjectivesEpidermolysis bullosa (EB) is a hereditary disorder characterized by mechanical stress‐induced blistering. The presence of extracutaneous complications such as cardiomyopathy and renal disease observed in severe EB subtypes and the fact that pruritus is a common symptom across all EB subtypes indicate that EB is not only a skin fragility disease but also a systemic inflammatory disorder. Our study aims to elucidate the basis of the systemic inflammation seen in EB patients.MethodsWe analyzed serum samples of 20 EB patients by Luminex bead‐based cytokine assays and enzyme‐linked immunosorbent assays.ResultsThe serum levels of sIL‐2R, IL‐6, HGF, M‐CSF, SCGF‐β, IL‐8, IL‐16, IFN‐γ, MIF, MIP‐1α, and thymic stromal lymphopoietin (TSLP) (p< .01,p< .01,p< .01,p< .01,p< .01,p< .05,p< .05,p< .05,p< .05,p< .05, andp= .01, respectively) were found to be significantly elevated in EB patients, whereas TNF‐β (p< .01) was decreased. Th2 cytokines including IL‐4, IL‐5, and IL‐13 were not elevated in EB patients. In contrast, TSLP was significantly increased, and IL‐31 and oncostatin M were not statistically significant but tended to be higher in the patients than in healthy controls. Among proinflammatory cytokines such as IL‐1β, IL‐6, and TNF‐α, IL‐6 was elevated in EB patients.ConclusionsThe imbalance of several itch mediators and proinflammatory cytokines was identified. Biologics targeting the cytokines found to be elevated in the sera of patients is considered as a beneficial treatment option for EB.
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