Sjögren syndrome or Sjögrens syndrome (SjS, SS) is a long-term autoimmune disease that affects the bodys moisture-producing (lacrimal and salivary) glands, and often seriously affects other organ systems, such as the lungs, kidneys, and nervous system. Primary symptoms are dryness (dry mouth and dry eyes), pain and fatigue. Other symptoms can include dry skin, vaginal dryness, a chronic cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. Those affected are also at an increased risk (15%) of lymphoma.While the exact cause is unclear, it is believed to involve a combination of genetics and an environmental trigger such as exposure to a virus or bacterium. It can occur independently of other health problems (primary Sjögrens syndrome) or as a result of another connective tissue disorder (secondary Sjögrens syndrome). Sjögrens syndrome may be associated with other autoimmune diseases, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) or systemic sclerosis. The inflammation that results progressively damages the glands. Diagnosis is by biopsy of moisture-producing glands and blood tests for specific antibodies. On biopsy there are typically lymphocytes within the glands.While Sjögrens syndrome is one of the most common auto-immune diseases, it has no specific and non-invasive diagnostic tests and treatment is directed at managing the persons symptoms. For dry eyes, artificial tears, medications to reduce inflammation, punctal plugs, or surgery to shut the tear ducts may be tried. For a dry mouth, chewing gum (preferably sugar-free), sipping water, or a saliva substitute may be used.
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.