B-cell hyperactivity in primary Sjögren's syndrome

Abstract

Primary Sjögren's syndrome (pSS) is characterized by mononuclear inflammatory infiltrates and IgG plasma cells in salivary and lacrimal glands which lead to irreversible destruction of the glandular tissue and is accompanied by sensation of dryness of mouth and eyes. B cells play a central role in the immunopathogenesis and exhibit signs of hyperactivity. Hyperactivity of B cells is the consequence of the coordinated and integrated action of stimulation of the B-cell receptor, CD40 and toll-like receptors in the presence of appropriate cytokines. As discussed, overexpression of type I IFN and BAFF on one hand and IL-6 and IL-21 on the other hand are critically involved in the enhanced plasma cell formation in pSS patients. Hyperactivity of B cells results in secretion of autoantibodies and production of various cytokines. These insights in the role of B cells in the pathogenetic process of pSS offer ample targets for successful therapeutical intervention in pSS.

Evaluation of possible factors affecting contrast sensitivity function in patients with primary Sjögren’s syndrome

“…Studies that have investigated the pathophysiological mechanisms of pSS have also revealed the significant effect of BAFF on the development of pSS (10,11) . Overproduction of BAFF in transgenic mice has been shown to lead to pathological findings resembling those observed in pSS (12) .…”

The value of rituximab treatment in primary Sjögren's syndrome

“…Dysfunction of exocrine glands is accompanied by periductal mononuclear infiltration of these glands, mainly by CD4 + T cells and B cells. Involvement of B cells in pSS pathogenesis is further illustrated by the presence of autoantibodies directed against SS-A/Ro and/or SS-B/La, elevated levels of rheumatoid factor (RF), hypergammaglobulinemia, elevated levels of Bruton's tyrosine kinase in B cells and a significantly increased risk of Non-Hodgkin B cell lymphoma, predominantly mucosa-associated lymphoid tissue (MALT) lymphoma [1,2].…”

Sjögren’s syndrome, should we sign?

“…[19,20] Chronic expression of type I IFN also may also influence the function of glandular tissue, for example by promoting apoptosis of the epithelial cells or loss of integrity of the epithelium and consequently xerostomia. Irrespective of the fact whether the pSS patients exhibit high type I or type II IFN activity (i.e.…”