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What is Sjogren’s Syndrome?
It is a chronic autoimmune disease where the body’s immune system attacks moisture producing glands, resulting in the deficient production of tears and saliva. This causes salivary and lacrimal glands to become inflamed, thus resulting in a dry mouth and dry eyes. Other symptoms include joint pain and fatigue. Around half the time, Sjogren’s is accompanied by other autoimmune connective tissue diseases such as rheumatoid arthritis, lupus and scleroderma.

What is the prevalence of Sjogren’s Syndrome?
Sjogren’s syndrome is the third most common rheumatic autoimmune disorder, behind only rheumatoid arthritis  and systemic lupus erythematosus.  It is most common amongst females, who account for 90% of all Sjogren Syndrome cases, and typically affects individuals between 40 and 60. 200K to 3M US cases per year. Few studies report the incidence of the syndrome varies between 3 and 6 per 100,000 per year.

How is Sjogren’s Syndrome diagnosed?
Sjogren’s can be diagnosed through a series of blood, eye and dental tests. A blood test can detect irregular blood cell levels, common antibodies found in Sjogren’s and evidence of inflamed tissue. The Anti-Nuclear Antibody test is a blood test that can be performed to pinpoint the presence of ANAs, a group of antibodies that react abnormally with the cell nucleus, and is found within 70% of all Sjogren patients. Eye tests are used to measure tear production, mainly through the Schirmer test, and detect any damage within the cornea. Dental examinations monitor salivary glands and saliva production over time.

Is there any specific gene/pathway in Sjogren’s Syndrome that has been identified?
Many Sjogren patients have been characterized with abnormal gene expression levels within their minor salivary glands (MSG). In numerous primary Sjogren syndrome patients, Type I interferon genes were overexpressed, while carbonic anhydrase II (the gene linked to salivary production) and Bcl-2-like 2 was downregulated. Primary SS can also result from the activation of NF-kB which causes inflammation and autoimmunity. In addition, its development can also be linked with HLA alleles DRB1*03 and DQB1*02, for patients containing anti-SSA and/or anti-SSB antibodies. Recently, a group of researchers discovered six new genes, IRF5, STAT4, CXCR5, TNIP1 and TNFAIP3, that were found to contribute to Sjogren’s as well.

How is Sjogren’s Syndrome treated?
Treatment for Sjogren’s syndrome primarily focuses on improving symptoms and preventing complications. Over the counter eye drops may be used to relieve dry eyes, and saliva substitutes and mouthwashes may be taken to relieve dry mouth.

Other medications may be prescribed as well, depending on the condition. This includes pilocarpine, which treats symptoms of dry eyes and dry mouth, and hydroxychloroquine, which slows the immune system’s attack on the tear and saliva glands.

Are there any clinical trials underway for Sjogren’s Syndrome?
Yes, currently, there are 5 clinical trials underway at NIH for Sjogren’s Syndrome. More information can be found here.

How can RareShare be helpful to Sjogren’s Syndrome patients and families?
The Sjogren’s Syndrome RareShare Community has 25 members. There are currently two discussions underway, helping to connect patients, health workers, caregivers and families interested in Sjogren’s Syndrome and providing them continual access to community resources.

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