Sjogrens Syndrome and Lymphoma Development

Abstract

Primary Sjögren's syndrome (pSS) is a systemic as well as an organ-specific autoimmune disease characterized by lymphocytic infiltration of the glandular epithelial tissue. It has been reported that pSS patients have a relatively increased risk for the development of lymphoma and various factors such as cytokine stimulation, environmental exposures and viral infections as well as genetic events may contribute to the development of lymphoma in pSS patients. Over the past few decades, numerous efforts have been undertaken to search for the relationship between lymphoma and pSS, for example advances in molecular biology for clonality analysis and well-linked register cohort studies for the predictive value of clinical, laboratory and histological findings. Despite this, mechanisms and prediction of lymphoma development in pSS patients still remain to be defined. In this review we have summarized the current knowledge concerning incidence and risk factors of lymphoma development in pSS patients. In addition, the most recent discoveries in the emergence and treatment of lymphoma in pSS patients and the possible mechanism of lymphoma development are also discussed.

Skewed Production of IL-6 and TGFβ by Cultured Salivary Gland Epithelial Cells from Patients with Sjögren's Syndrome

“…Local IFNγ production in the salivary glands may perpetuate inflammation by inducing SGE cells to express HLA-DR, co-stimulatory molecules, cytokines and chemokines, leading to secretory gland dysfunction [2]. We therefore comprehensively examined the effects of IFNγ on the functions of SGE cells obtained from SS patients.…”

“…Because the salivary glands of SS patients contain T cells that express IFNγ and Stat1 mRNA [24] , with these T cells being predominantly Th1 cells [25] , the proinflammatory cytokine IFNγ has been considered a principle mediator of inflammation in SS patients, similar to TNFα in patients with RA [3] . Local IFNγ production in the salivary glands may perpetuate inflammation by inducing SGE cells to express HLA-DR, co-stimulatory molecules, cytokines and chemokines, leading to secretory gland dysfunction [2] . We therefore comprehensively examined the effects of IFNγ on the functions of SGE cells obtained from SS patients.…”

“…Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration into the salivary and lacrimal glands [1] , [2] . This chronic inflammation leads to destruction of the salivary glands and may ultimately result in salivary hypofunction.…”

Different serum levels of IgG and complements and recurrence rates in IgG4-positive and negative lacrimal gland benign lymphoepithelial lesion

“…This may indicate that levels of estrogen in female patients gradually with age, increasing the incidence of LGBLEL. IgG4-ROD may be associated with immune system diseases, such as allergic rhinitis, asthma, lymph node enlargement, and Sjӧgren's syndrome [16][17] . In our study, LGBLEL had a history of immune system disease, among which allergic rhinitis, sinusitis, and lymph node enlargement were common.…”