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Disease Profile

Sjogren syndrome

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

#N/A

ICD-10

#N/A

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Sicca syndrome; Sjogren-Gougerot syndrome

Summary

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]

Symptoms

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.

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

Cause

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]

Treatment

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.

Organizations

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

      Learn more

      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.
      • MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
      • Genetics Home Reference contains information on Sjogren syndrome. This website is maintained by the National Library of Medicine.
      • 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.

          References

          1. NINDS Sjögren's Syndrome Information Page. National Institute of Neurological Disorders and Stroke (NINDS). October 15, 2012; https://www.ninds.nih.gov/disorders/sjogrens/sjogrens.htm.
          2. Sjögren syndrome. Genetics Home Reference (GHR). August 2013; https://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; https://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. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3616867/.
          5. Sjogren Syndrome. Medscape Reference. July 9, 2014; https://emedicine.medscape.com/article/332125-overview#showall.

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