B cell subsets and dysfunction of regulatory B cells in IgG4-related diseases and primary Sjögren's syndrome: the similarities and differences.
B cell subsets and dysfunction of regulatory B cells in IgG4-related diseases and primary Sjögren's syndrome: the similarities and differences.
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DOI：
10.1186/ar4571
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发表时间：
2014-05-29
影响因子：
4.9
通讯作者：
Zhang F
中科院分区：
文献类型：
--
作者：
Lin W;Jin L;Chen H;Wu Q;Fei Y;Zheng W;Wang Q;Li P;Li Y;Zhang W;Zhao Y;Zeng X;Zhang F
IgG4相关疾病（IgG4-RD）是一种涉及多系统的自身免疫性疾病。异常激活和分化的 B 细胞可能发挥重要作用。调节性 B 细胞 (Breg) 是新定义的具有免疫抑制功能的 B 细胞亚群。在本研究中，我们研究了 IgG4-RD 患者、原发性干燥综合征 (pSS) 以及健康对照 (HC) 中 B 细胞亚群的差异、B 细胞上共刺激分子的表达以及 Breg 细胞的功能。纳入新诊断的 IgG4-RD 患者（n = 48），招募 38 名未经治疗的 pSS 患者和 30 名健康志愿者作为疾病和健康对照。为了分析 B 细胞亚群和 B 细胞活性，对 PBMC 进行表面染色并通过流式细胞术进行检测。通过将分离的CD19++CD24hiCD38hi Breg细胞与纯化的CD4++CD25-T细胞共培养来测试Breg细胞的功能。通过ELISA和细胞计数珠阵列测量血清细胞因子。还分析了临床数据和实验室结果之间的关系。与pSS患者和HC相比，IgG4-RD患者外周Breg细胞的频率较低。有趣的是，IgG4-RD 患者外周 B 细胞中 CD19+CD24-CD38hi B 细胞亚群显着高于 pSS 患者和 HC，这与血清 IgG4 水平相关。 IgG4-RD患者B细胞上BAFF-R和CD40的表达显着低于pSS患者和HC。与 HC 不同，pSS 患者的 Breg 细胞缺乏抑制功能。 IgG4-RD 和 pSS 患者的 B 细胞表现出多种异常，包括 B 细胞亚群紊乱、关键信号分子、共刺激分子和炎症细胞因子的异常表达。此外，显着增加的B细胞亚群CD19 + CD24-CD38hi B细胞可能在IgG4-RD的发病机制中发挥重要作用。
IgG4-related disease (IgG4-RD) is a multisystem-involved autoimmune disease. Abnormally activated and differentiated B cells may play important roles. Regulatory B cells (Breg) are newly defined B cell subgroups with immunosuppressive functions. In this study, we investigated the differences of B cell subsets, the expressions of co-stimulatory molecules on B cells, and the function of Breg cells in patients with IgG4-RD, primary Sjögren’s syndrome (pSS) as well as in healthy controls (HC). Newly diagnosed IgG4-RD patients (n = 48) were enrolled, 38 untreated pSS patients and 30 healthy volunteers were recruited as disease and healthy controls. To analyze B cell subsets and B cell activity, PBMCs were surface stained and detected by flow cytometry. The function of Breg cells was tested by coculturing isolated CD19 + CD24hiCD38hi Breg cells with purified CD4 + CD25- T cells. Serum cytokines were measured by ELISA and cytometric bead array. Relationship between clinical data and laboratory findings were analyzed as well. Compared with pSS patients and HC, IgG4-RD patients had a lower frequency of peripheral Breg cells. Interestingly, CD19 + CD24-CD38hi B cell subsets were significantly higher in peripheral B cells from IgG4-RD patients than in pSS patients and HC, which correlated with serum IgG4 levels. The expression of BAFF-R and CD40 on B cells was significantly lower in IgG4-RD patients compared with those in pSS patients and HC. Unlike HC, Breg cells from pSS patients lacked suppressive functions. B cells in patients with IgG4-RD and pSS display a variety of abnormalities, including disturbed B cell subpopulations, abnormal expression of key signaling molecules, co-stimulatory molecules, and inflammatory cytokines. In addition, a significantly increased B cell subset, CD19 + CD24-CD38hi B cells, may play an important role in the pathogenesis of IgG4-RD.
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一种新的临床实体，IgG4 相关疾病 (IgG4RD)：一般概念和细节。
影响因子：
2.2
作者：
Umehara, Hisanori;Okazaki, Kazuichi;Masaki, Yasufumi;Kawano, Mitsuhiro;Yamamoto, Motohisa;Saeki, Takako;Matsui, Shoko;Sumida, Takayuki;Mimori, Tsuneyo;Tanaka, Yoshiya;Tsubota, Kazuo;Yoshino, Tadashi;Kawa, Shigeyuki;Suzuki, Ritsuro;Takegami, Tsutomu;Tomosugi, Naohisa;Kurose, Nozomu;Ishigaki, Yasuhito;Azumi, Atsushi;Kojima, Masaru;Nakamura, Shigeo;Inoue, Dai
通讯作者：
Inoue, Dai
DOI：
10.1111/j.1749-6632.2009.04651.x
发表时间：
2009-01-01
期刊：
CONTEMPORARY CHALLENGES IN AUTOIMMUNITY
影响因子：
--
作者：
Lemoine, Sebastien;Morva, Ahsen;Jamin, Christophe
通讯作者：
Jamin, Christophe
影响因子：
64.5
作者：
KUCHROO, VK;DAS, MP;GLIMCHER, LH
通讯作者：
GLIMCHER, LH
影响因子：
3.5
作者：
Mann MK;Ray A;Basu S;Karp CL;Dittel BN
通讯作者：
Dittel BN
影响因子：
20.3
作者：
Li, Xiaojuan;Zhong, Hui;Yazdanbakhsh, Karina
通讯作者：
Yazdanbakhsh, Karina