B cell subsets and dysfunction of regulatory B cells in IgG4-related diseases and primary Sjögren’s syndrome: the similarities and differencesReportar como inadecuado

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Arthritis Research and Therapy

, 16:R118

First Online: 29 May 2014Received: 23 September 2013Accepted: 12 May 2014


IntroductionIgG4-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.

MethodsNewly 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 + CD24CD38 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.

ResultsCompared with pSS patients and HC, IgG4-RD patients had a lower frequency of peripheral Breg cells. Interestingly, CD19 + CD24-CD38 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.

ConclusionsB 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-CD38 B cells, may play an important role in the pathogenesis of IgG4-RD.


BAFFB-cell activating factor

Bregregulatory B cells

CBAcytometric bead array

CRPC-reactive protein

ELISAenzyme-linked immunosorbent assay

ESRerythrocyte sedimentation rate

FACSfluorescence-activated cell sorter

FCSfetal calf serum

HChealthy controls



IgG4-RDIgG4-related disease


mAbsmonoclonal antibodies

MFImedian fluorescence intensity

PBMCperipheral blood mononuclear cell

pSSprimary Sjögren’s syndrome

RPMIRoswell Park Memorial Institute

SEMstandard error of the mean

TACItransmembrane activator and calcium modulator

Teffeffector T cells

TGFtransforming growth factor

ThT helper

TNFtumor necrosis factor.

Electronic supplementary materialThe online version of this article doi:10.1186-ar4571 contains supplementary material, which is available to authorized users.

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Autor: Wei Lin - Lixia Jin - Hua Chen - Qingjun Wu - Yunyun Fei - Wenjie Zheng - Qian Wang - Ping Li - Yongzhe Li - Wen Zhang -

Fuente: https://link.springer.com/

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