Derailed B cell homeostasis in patients with mixed connective tissue disease

Abstract 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. U...

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Bibliographic Details
Published in:Human immunology 2013-07, Vol.74 (7), p.833-841
Main Authors: Hajas, A, Barath, S, Szodoray, P, Nakken, B, Gogolak, P, Szekanecz, Z, Zold, E, Zeher, M, Szegedi, G, Bodolay, E
Format: Article
Language:English
Subjects:
Adrenal Cortex Hormones - administration & dosage
Allergy and Immunology
Antigens, CD - metabolism
Autoantibodies - blood
B-Lymphocyte Subsets - drug effects
B-Lymphocyte Subsets - immunology
Cells, Cultured
Cyclophosphamide - administration & dosage
Disease Progression
Homeostasis - drug effects
Humans
Immunoglobulin Class Switching
Immunologic Memory
Immunophenotyping
Methotrexate - administration & dosage
Mixed Connective Tissue Disease - drug therapy
Mixed Connective Tissue Disease - immunology
Plasma Cells - drug effects
Plasma Cells - immunology
Ribonucleoprotein, U1 Small Nuclear - immunology
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Summary:Abstract 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 + CD38high ); (2) naive B cells (CD19 + CD27-IgD + CD38low ); (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 + CD27high 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 CD27high 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.
ISSN:0198-8859
1879-1166
DOI:10.1016/j.humimm.2013.04.007