This increases the frequency of oncogenic mutation, leading to any dysfunction at checkpoints of autoimmune B-cell activation to transform into malignancy. A studys finding has concluded the continuous stimulation of autoimmune B cells, leading to subtle germinal abnormalities in genes having specific consequences in B cells, which underlies the susceptibility to lymphoma.Apart from this notably higher incidence of malignant NHL, Sjögrens patients show only modest or clinically insignificant deterioration in specific organ-related function, which explains the only slight increases in mortality rates of Sjögrens patients in comparison with the remainder of the population.Sjögrens syndrome is associated with a high burden of illness, and has been shown to markedly reduce quality of life (QoL), with a significant impact on ability to work resulting from increased rates of disability. The reduction in QoL is similar to that seen in other chronic conditions such as rheumatoid arthritis, lupus and fibromyalgia. Epidemiology
Sjögrens syndrome (SS) is the second-most common rheumatic autoimmune disorder, behind rheumatoid arthritis and systemic lupus erythematosus. There are no geographical differences in the rates of SS. Sjögrens syndrome has been reported in all areas of the world, although regional rates have not been well studied. Depending on the criteria for determining prevalence, studies estimate the prevalence of SS at between 500,000 and two million people in the United States. Broader studies of SS prevalence range widely, with some reports of up to a prevalence of 3% of the population.
Environmental and hormonal factors, in concert with an appropriate genetic background, are believed to trigger Sjögrens syndrome, which dysregulates epithelial cells and allows aberrant homing and activation of dendritic cells (DCs), T cells, and B cells. Dendritic cells are antigen-presenting cells  that process antigen material and present it to other T cells. Following the migration of lymphocytes into the glands in response to chemokines and specific adhesion molecules, T cells interact with epithelial cells. Epithelial cells are further activated by proinflammatory cytokines (IL-1β, IFN-γ, and TNF), which are produced by adjacent T cells. The early accumulation of plasmacytoid dendritic cells in the target tissues, which produce high levels of type 1 IFNs, seems important, as these cells can further dysregulate the immune response through abnormal retention of lymphocytes in the tissues and their subsequent activation. IFN-α stimulates the production of B-cell activating factor (BAFF) by epithelial cells, DCs, and T cells. BAFF stimulates aberrant B-cell maturation, leading to the emergence of self-reactive B cells, which locally produce autoantibodies, in a germinal centre-like structure (GC-like), which is also the location of lymphomagenesis (origin of lymphoma). Programmed cell death
Dysregulation of apoptosis (programmed cell death) is believed to play a role in the pathogenesis of a variety of autoimmune diseases, though its role in Sjögrens syndrome is controversial. Both the Fas and Fas ligand proteins are overexpressed in primary Sjögrens patients, while expression of BCL-1, which is known to downregulate apoptosis, was found significantly reduced in acinar and ductal epithelial cells of Sjögrens patients compared to healthy people.