Derailed B cell homeostasis in patients with mixed connective tissue disease
Derailed B cell homeostasis in patients with mixed connective tissue disease
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DOI：
10.1016/j.humimm.2013.04.007
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发表时间：
2013-07-01
期刊：
影响因子：
2.7
通讯作者：
Bodolay, E.
中科院分区：
文献类型：
--
作者：
Hajas, A.;Barath, S.;Bodolay, E.
混合性结缔组织病（MCTD）是一种全身性自身免疫性疾病，其特征在于存在针对U1-RNP蛋白的抗体。我们的目的是确定MCTD患者外周B细胞亚群的表型异常。从46名MCTD患者和20名对照中获得血液样品。应用抗CD 19、抗CD 27、抗IgD和抗CD 38单克隆抗体，通过流式细胞术鉴定以下B细胞亚群：（1）过渡型B细胞（CD 19 + CD 27-IgD + CD 38（高））;（2）初始B细胞（CD 19 + CD 27-IgD + CD 38（低））;（3）非转换记忆B细胞（4）转换记忆B细胞（5）双阴性（DN）记忆B细胞（CD 19 + CD 27-IgD-）和（6）浆细胞（CD 19 + CD 27（高）IgD-）。MCTD组中过渡型B细胞、幼稚型B细胞和DN型B细胞比例均高于对照组。DN B细胞表面标志CD 95阳性。这种记忆B细胞群与疾病活动性密切相关。浆细胞数量也增加，且浆细胞数量与抗U1 RNP水平相关。环磷酰胺、甲氨蝶呤和皮质类固醇治疗可减少DN和CD 27（高）B细胞的数量。总之，在MCTD的外周血B细胞亚群中发现了几种异常，这加强了脱轨的体液自身免疫过程在发病机制中的作用。(c)2013年美国组织相容性和免疫遗传学学会。爱思唯尔公司出版All rights reserved.
Mixed connective tissue disease (MCTD) is a systemic autoimmune disorder, characterized by the presence of antibodies to U1-RNP protein. We aimed to determine phenotypic abnormalities of peripheral B cell subsets in MCTD. Blood samples were obtained from 46 MCTD patients, and 20 controls. Using anti-CD19, anti-CD27, anti-IgD and anti-CD38 monoclonal antibodies, the following B cell subsets were identified by flow cytometry: (1) transitional B cells (CD19 + CD27-IgD + CD38(high)); (2) naive B cells (CD19 + CD27-IgD + CD38(low)); (3) non-switched memory B cells (CD19 + CD27 + IgD+); (4) switched memory B cells (CD19 + CD27 + IgD-); (5) double negative (DN) memory B cells (CD19 + CD27-IgD-) and (6) plasma cells (CD19 + CD27(high)IgD-). The proportion of transitional B cells, naive B cells and DN B lymphocytes was higher in MCTD than in controls. The DN B cells were positive for CD95 surface marker. This memory B cells population showed a close correlation with disease activity. The number of plasma cells was also increased, and there was an association between the number of plasma cells and the anti-U1RNP levels. Cyclophosphamide, methotrexate, and corticosteroid treatment decreased the number of DN and CD27(high) B cells. In conclusion, several abnormalities were found in the peripheral B-cell subsets in MCTD, which reinforces the role of derailed humoral autoimmune processes in the pathogenesis. (c) 2013 American Society for Histocompatibility and Immunogenetics. Published by Elsevier Inc. All rights reserved.