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An important role for B-cell activation factor and B cells in the pathogenesis of Sjogren's syndrome


PURPOSE OF REVIEW: This review provides an update on the specific, strong association between dysregulated production of the cytokine B-cell activation factor and Sjogren's syndrome, and offers new perspectives on potential pathogenic mechanisms. RECENT FINDINGS: Excess B-cell activation factor in mice triggers Sjogren's syndrome-like symptoms, and elevated serum B-cell activation factor in humans correlates with Sjogren's syndrome. B-cell activation factor is produced locally by activated monocytes, T cells and dendritic cells, and by epithelial cells and infiltrating B cells. Moreover, recent data in humans suggest that the innate immune system plays a role as an initiator of immune disorders in inflamed tissues. SUMMARY: Recent data have demonstrated the critical role of B-cell activation factor and B cells in the pathogenesis of Sjogren's syndrome, and its association with B lymphomas. Moreover, B-cell depleting treatments have confirmed the critical role of B cells in Sjogren's syndrome. Excess B-cell activation factor possibly corrupts B-cell tolerance and allows the emergence of self-reactive B cells that efficiently present antigen to T cells. In addition, B-cell activation factor may stimulate T-cell independent activation of B cells via Toll-like receptors; this recently identified mechanism could also play a separate, detrimental role in autoimmunity.

Type Journal
ISBN 1040-8711 (Print)
Authors Mackay, F.;Groom, J. R.;Tangye, S. G. :
Published Date 2007-01-01 00:00:00
Published Volume 19
Published Issue 5
Published Pages 406-13
Status Published In-print
OpenAccess Link MackayF COR.pdf