Significant decrease in peripheral regulatory B cells is an immunopathogenic feature of dermatomyositis.
Significant decrease in peripheral regulatory B cells is an immunopathogenic feature of dermatomyositis.
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外周调节性 B 细胞显着减少是皮肌炎的免疫致病特征
DOI：
10.1038/srep27479
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发表时间：
2016-06-07
影响因子：
4.6
通讯作者：
Wang G
中科院分区：
文献类型：
--
作者：
Li W;Tian X;Lu X;Peng Q;Shu X;Yang H;Li Y;Wang Y;Zhang X;Liu Q;Wang G
调节性B细胞(Bregs)在维持自身耐受性方面至关重要。它们在皮肌炎(DM)中的作用尚不清楚，DM是一种自身免疫性疾病，其特征是对过度激活的B和T细胞进行不适当的调节。在本研究中，我们对30例糖尿病患者、37例疾病对照和23例健康对照的血液标本中CD19+CD24HighCD38HighBreg亚群进行了流式细胞术分析。与疾病对照组(p<； 0.0001)和健康对照组(p<； 0.0001)相比，DM患者Bregs的发生率显著降低。糖尿病患者Bregs缺乏症(本研究中定义为Bregs/B细胞 <； 0.50%)的患病率高达73.3%。此外，肌炎自身抗体阳性的糖尿病患者的Bregs水平通常低于抗体阴性的患者(p= 0.036)，而有间质性肺疾病的DM患者的Bregs水平也低于不伴有间质性肺疾病的DM患者(p= 0.041)。在一项随访研究中，7名DM患者被认为处于缓解期，治疗后他们的BREG水平显著上升(p= 0.022)。我们的研究揭示了Breg缺陷是DM的一种免疫致病特征，并为DM临床干预的新的免疫治疗靶点的设计提供了见解。
Regulatory B cells (Bregs) are critical in maintaining self-tolerance. Their role in dermatomyositis (DM), an autoimmune disease characterized by inappropriate regulation of hyperactivated B and T cells, has not been clearly defined. In the current study, we performed flow cytometry analysis of studied CD19+CD24highCD38highBreg subpopulations in blood samples from 30 patients with DM, 37 diseased controls and 23 healthy controls. A significant decrease was observed in the frequency of Bregs in DM patients compared to that in diseased controls (p< 0.0001) and in healthy controls (p< 0.0001). And the prevalence of Bregs deficiency (defined as Bregs/B cells < 0.50% in this study) in DM patients went as high as 73.3%. Furthermore, DM patients with positive myositis specific autoantibody often had lower Bregs levels than negative patients (p= 0.036) and lower level of Bregs was also found in DM patients with interstitial lung disease than in DM patients without (p= 0.041). In a follow-up study, seven DM patients were considered to be in remission stage and their Breg levels were found to have significantly increased after treatment (p= 0.022). Our research revealed that Breg deficiency is an immunopathogenic feature of DM and provided insights into the design of new immunotherapy target for DM clinical interventions.