ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 924

B Cell Subsets and Dysfunction Of Regulatory B Cells In IgG4-Related Diseases and Primary Sjögren Syndrome: The Similarities and Differences

Wei Lin1, Lixia Jin2, Wen Zhang1, Hua Chen3, QingJun Wu4, Yunyun Fei1, Yan Zhao1, Xiaofeng Zeng5 and Fengchun Zhang3, 1Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China, 2Tsinghua University, Beijing, China, 3Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China, 4Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China, 5Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: , ,

Session Information

Title: B cells in Human and Animal Arthritis

Session Type: Abstract Submissions (ACR)

Background/Purpose: IgG4 related disease (IgG4-RD) is a chronic, 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 the peripheral blood of patients with IgG4-RD, primary Sjögren’s syndrome (pSS), and healthy donors. 

Methods: We recruited 38 newly diagnosed IgG4-RD patients, 38 newly diagnosed pSS patients and 30 healthy volunteers were included as disease and healthy controls. To analyze B cell subsets and B cell activity, PBMCs were surface stained with CD19, CD24, CD38, BAFF-R, CD40, CD80 and CD86 mAbs and then detected by flow cytometry. The function of Bregs was tested by co-culturing of isolated CD19+CD24hiCD38hi Breg cells with purified CD4+CD25- effector T cells.  Serum cytokines were measured by ELISA. Correlation of clinical data and laboratory findings were measured as well.

Results: Compared with pSS patients and healthy controls, IgG4-RD patients had a lower frequency of peripheral mature B cells and Breg cells. Interestingly, CD19+CD24-CD38hi B cell subsets were significantly higher in peripheral blood B cells from new-onset IgG4-RD patients than in pSS patients and healthy controls. The expression of BAFF-R and CD40 on B cells was significantly lower in IgG4-RD patients compared with those in pSS patients and healthy controls. Whereas, the expression of CD86 and CD80 on B cells was significantly increased in IgG4-RD patients compared with those in pSS patients and healthy controls. Unlike healthy B cells, CD19+CD24hiCD38hi Breg cells from pSS patients were lack of suppression function.

Conclusion: 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. A significantly increased B cell subset, CD19+CD24-CD38hi B cells may play an important role in the pathogenesis of IgG4-RD.

Disclosure:

W. Lin,
None;

L. Jin,
None;

W. Zhang,
None;

H. Chen,
None;

Q. Wu,
None;

Y. Fei,
None;

Y. Zhao,
None;

X. Zeng,
None;

F. Zhang,
None.

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