National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Sjogren syndrome

Not a rare disease Not a rare disease
Other Names:
Sicca syndrome; Sjogren-Gougerot syndrome
Sjögren syndrome is an autoimmune disorder in which immune cells attack and destroy the glands that produce tears and saliva. Sjögren syndrome is also associated with rheumatic disorders such as rheumatoid arthritis or systemic lupus erythematosus. The hallmark symptoms of Sjögren syndrome are dry mouth and dry eyes. In addition, Sjogren syndrome may cause skin, nose, and vaginal dryness. It also may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain.[1][2] Treatment focuses on the symptoms in each person and may include moisture replacement therapies, nonsteroidal anti-inflammatory drugs and, in severe cases, corticosteroids or immunosuppressive drugs.[1]
Last updated: 1/21/2016

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms Other Names
Learn More:
HPO ID
Percent of people who have these symptoms is not available through HPO
Abnormality of metabolism/homeostasis
Laboratory abnormality
Metabolism abnormality
[ more ] 		0001939
Autoimmunity
Autoimmune disease
Autoimmune disorder
[ more ] 		0002960
Autosomal recessive inheritance 0000007
Keratoconjunctivitis sicca
Dry eyes
0001097
Rheumatoid arthritis 0001370
Tubulointerstitial nephritis 0001970
Xerostomia
Dry mouth
Dry mouth syndrome
Reduced salivation
[ more ] 		0000217
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Last updated: 7/1/2020
Sjogren syndrome likely results from a combination of genetic and environmental factors (it appears to be multifactorial). Several different genes appear to affect the risk of developing the condition, but specific genes have not been confirmed. Simply having one of these genes does not cause a person to develop the disease. Some sort of "trigger" is also needed, such as a viral or bacterial infection. The genetic variations that increase susceptibility may reduce the body's ability to turn off the immune response when it is no longer needed.[2][3] The possibility that the endocrine and nervous systems may play a role in the disease is also being studied.[3]
Last updated: 1/21/2016
A genetic predisposition to Sjogren syndrome has been suggested, but the condition itself does not appear to be inherited.[2][4] This means that a person might inherit an increased risk to develop the condition. Familial clustering of different autoimmune diseases, as well as co-association of multiple autoimmune diseases in individuals, have frequently been reported. Some studies have shown that up to 30% of people with Sjogren syndrome have relatives with autoimmune diseases.[4] While the relatives of people with Sjogren syndrome are at an increased risk of developing autoimmune diseases in general, they are not necessarily more likely to develop Sjogren syndrome.[2][4]
Last updated: 1/21/2016

FDA-Approved Treatments

The medication(s) listed below have been approved by the Food and Drug Administration (FDA) as orphan products for treatment of this condition. Learn more orphan products.

Most people with Sjogren syndrome have limited symptoms such as dry eyes and dry mouth. Their general health and life expectancy are largely unaffected.[2] Increased mortality may be related to conditions associated with Sjogren syndrome, such as systemic lupus erythematous (SLE), rheumatoid arthritis (RA), or primary biliary cirrhosis. People with Sjogren syndrome who don't develop a lymphoproliferative disorder can generally expect to have a normal life span.[5]
Last updated: 10/17/2014

If you need medical advice, you can look for doctors or other healthcare professionals who have experience with this disease. You may find these specialists through advocacy organizations, clinical trials, or articles published in medical journals. You may also want to contact a university or tertiary medical center in your area, because these centers tend to see more complex cases and have the latest technology and treatments.

If you can’t find a specialist in your local area, try contacting national or international specialists. They may be able to refer you to someone they know through conferences or research efforts. Some specialists may be willing to consult with you or your local doctors over the phone or by email if you can't travel to them for care.

You can find more tips in our guide, How to Find a Disease Specialist. We also encourage you to explore the rest of this page to find resources that can help you find specialists.

Healthcare Resources

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

  • ClinicalTrials.gov lists trials that are related to Sjogren syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

    Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

  • The Autoimmune Registry supports research for Sjogren syndrome by collecting information about patients with this and other autoimmune diseases. You can join the registry to share your information with researchers and receive updates about participating in new research studies. Learn more about registries.

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

Organizations Providing General Support

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference contains information on Sjogren syndrome. This website is maintained by the National Library of Medicine.
  • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
  • The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.
  • The National Institute of Dental and Craniofacial Research (NIDCR), purposes to improve oral, dental and craniofacial health through research, research training, and the dissemination of health information. Click on the link to view information on this topic. 
  • The National Institute of Neurological Disorders and Stroke (NINDS) collects and disseminates research information related to neurological disorders. Click on the link to view information on this topic.

In-Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • MeSH® (Medical Subject Headings) is a terminology tool used by the National Library of Medicine. Click on the link to view information on this topic.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Sjogren syndrome. Click on the link to view a sample search on this topic.

Other Conferences

  • !LINK!, February 3, 2013 - February 8, 2013
    Location: Hotel Galvez, Galveston, TX
    Description: The overall goal of this conference is to expedite progress in research in the area of salivary and exocrine tissue biology and to use research findings for the development of new therapies for salivary and other exocrine tissues dysfunctions. A secondary goal is to facilitate collaborations among salivary researchers and investigators in complementary basic, translational and clinical fields.

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

  1. NINDS Sjögren's Syndrome Information Page. National Institute of Neurological Disorders and Stroke (NINDS). October 15, 2012; http://www.ninds.nih.gov/disorders/sjogrens/sjogrens.htm.
  2. Sjögren syndrome. Genetics Home Reference (GHR). August 2013; http://ghr.nlm.nih.gov/condition/sjogren-syndrome.
  3. Questions and Answers about Sjögren’s Syndrome. National Institute of Arthritis and Musculoskeletal and Skin Diseases. June 2013; http://www.niams.nih.gov/Health_Info/Sjogrens_Syndrome/default.asp.
  4. Tincani A, Andreoli L, Cavazzana I, Doria A, Favero M, Fenini MG, Franceschini F, Lojacono A, Nascimbeni G, Santoro A, Semeraro F, Toniati P, Shoenfeld Y. Novel aspects of Sjögren's syndrome in 2012. BMC Med. 2013 Apr 4; 11:93. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3616867/.
  5. Sjogren Syndrome. Medscape Reference. July 9, 2014; http://emedicine.medscape.com/article/332125-overview#showall.