Document Detail


B-cell tolerance breakdown in Sjögren's syndrome: focus on BAFF.
MedLine Citation:
PMID:  20457281     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Sjögren's syndrome (SS) is an autoimmune epithelitis hallmarked by a destruction of epithelial cells, the subsequent lymphocytic infiltration of exocrine glands and their ensuing dryness. Given the prominent role currently assigned to B cells in SS, it is not surprising that the B cell activating factor belonging to the Tumor Necrosis Factor family (BAFF) is involved in its pathogenesis. When overexpressed, this cytokine leads to self-reactive B cells emergence at the transitional B-cell stage. BAFF overexpression that has been observed in SS, is associated with B-cell tolerance breakdown and autoantibody production. Furthermore, BAFF is responsible for the abnormal distribution of B cells subsets and B-cell hyperactivity described in the blood. In the salivary glands, a minority of B-cell clusters represent ectopic germinal center cells, while the majority manifest features consistent with transitional type 2 (T2) and marginal-zone (MZ)-like B cells. Interestingly, both types of B-cell cluster include autoreactive B cells and BAFF is associated with expansion of T2 B cells and MZ-like B cells in the salivary glands. Finally, BAFF plays a major role in B-cell repopulation after their depletion by rituximab in SS.
Authors:
Marie-Michèle Varin; Laëtitia Le Pottier; Pierre Youinou; Damien Saulep; Fabienne Mackay; Jacques-Olivier Pers
Publication Detail:
Type:  Journal Article; Review     Date:  2010-05-08
Journal Detail:
Title:  Autoimmunity reviews     Volume:  9     ISSN:  1873-0183     ISO Abbreviation:  Autoimmun Rev     Publication Date:  2010 Jul 
Date Detail:
Created Date:  2010-07-12     Completed Date:  2011-01-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101128967     Medline TA:  Autoimmun Rev     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  604-8     Citation Subset:  IM    
Affiliation:
EA 2216 Immunologie et Pathologie, Université de Brest, France.
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MeSH Terms
Descriptor/Qualifier:
Animals
Autoantibodies / metabolism
B-Cell Activating Factor / genetics,  immunology*
B-Lymphocytes / immunology*,  metabolism,  pathology*
Germinal Center / immunology,  metabolism,  pathology
Humans
Immune Tolerance*
Mice
Mice, Transgenic
Precursor Cells, B-Lymphoid
Salivary Glands / immunology,  metabolism,  pathology
Sjogren's Syndrome / etiology,  immunology*,  pathology*
Chemical
Reg. No./Substance:
0/Autoantibodies; 0/B-Cell Activating Factor

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