Sjögren syndrome is a disorder whose main features are dry eyes and a dry mouth. The condition typically develops gradually beginning in middle adulthood but can occur at any age.
Sjögren syndrome is classified as an autoimmune disorder, one of a large group of conditions that occur when the immune system attacks the body's own tissues and organs. In Sjögren syndrome, the immune system primarily attacks the glands that produce tears (the lacrimal glands) and saliva (the salivary glands), impairing the glands' ability to secrete these fluids.
Dry eyes may lead to itching, burning, a feeling of sand in the eyes, blurry vision, or intolerance of bright or fluorescent lighting. A dry mouth can feel chalky or full of cotton, and affected individuals may have difficulty speaking, tasting food, or swallowing. Because saliva helps protect the teeth and the tissues of the oral cavity, people with Sjögren syndrome are at increased risk of tooth decay and infections in the mouth.
In most people with Sjögren syndrome, dry eyes and dry mouth are the primary features of the disorder, and general health and life expectancy are largely unaffected. However, in some cases the immune system also attacks and damages other organs and tissues. This complication is known as extraglandular involvement. Affected individuals may develop inflammation in connective tissues, which provide strength and flexibility to structures throughout the body. Disorders involving connective tissue inflammation are sometimes called rheumatic conditions. In Sjögren syndrome, extraglandular involvement may result in painful inflammation of the joints and muscles; dry, itchy skin and skin rashes; chronic cough; a hoarse voice; kidney and liver problems; numbness or tingling in the hands and feet; and, in women, vaginal dryness. Prolonged and extreme tiredness (fatigue) severe enough to affect activities of daily living may also occur in this disorder. A small number of people with Sjögren syndrome develop lymphoma, a blood-related cancer that causes tumor formation in the lymph nodes but can spread to other organs.
Some individuals who are first diagnosed with another rheumatic disorder, such as rheumatoid arthritis or systemic lupus erythematosus, later develop the dry eyes and dry mouth characteristic of Sjögren syndrome. Other autoimmune disorders can also develop after the onset of Sjögren syndrome. In all, about half of all individuals with Sjögren syndrome also have another autoimmune disorder.
Sjögren syndrome is a relatively common disorder; it occurs in 0.1 to 1 percent of the population. It is difficult to determine the exact prevalence because the characteristic features of this disorder, dry eyes and dry mouth, can also be caused by many other conditions. Women develop Sjögren syndrome about 10 times more often than men; the specific reason for this difference is unknown but likely involves the effects of sex hormones on immune system function.
Sjögren syndrome is thought to result from a combination of genetic and environmental factors; however, no associations between specific genetic changes and the development of Sjögren syndrome have been confirmed. Researchers believe that variations in many genes affect the risk of developing Sjögren syndrome, but that development of the condition may be triggered by something in the environment. In particular, viral or bacterial infections, which activate the immune system, may have the potential to encourage the development of Sjögren syndrome in susceptible individuals. The genetic variations that increase susceptibility may reduce the body's ability to turn off the immune response when it is no longer needed.
A predisposition to develop autoimmune disorders can be passed through generations in families. Relatives of people with Sjögren syndrome are at an increased risk of developing autoimmune diseases, although they are not necessarily more likely to develop Sjögren syndrome in particular. The inheritance pattern of this predisposition is unknown.
Other Names for This Condition
- Dacryosialoadenopathia atrophicans
- Gougerot-Houwer-Sjogren syndrome
- Gougerot-Sjogren syndrome
- Keratoconjunctivitis sicca
- Keratoconjunctivitis sicca-xerostomia
- Sicca syndrome
- Sjogren's syndrome
- Sjogren-Gougerot syndrome
Additional Information & Resources
Genetic and Rare Diseases Information Center
Research Studies from ClinicalTrials.gov
Catalog of Genes and Diseases from OMIM
Scientific Articles on PubMed
- Basiaga ML, Stern SM, Mehta JJ, Edens C, Randell RL, Pomorska A, Irga-Jaworska N, Ibarra MF, Bracaglia C, Nicolai R, Susic G, Boneparth A, Srinivasalu H, Dizon B, Kamdar AA, Goldberg B, Knupp-Oliveira S, Antón J, Mosquera JM, Appenzeller S, O'Neil KM, Protopapas SA, Saad-Magalhães C, Akikusa JD, Thatayatikom A, Cha S, Nieto-González JC, Lo MS, Treemarcki EB, Yokogawa N, Lieberman SM; Childhood Arthritis and Rheumatology Research Alliance and the International Childhood Sjögren Syndrome Workgroup. Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria. Rheumatology (Oxford). 2021 Jul 1;60(7):3144-3155. doi: 10.1093/rheumatology/keaa757. Citation on PubMed
- Bayetto K, Logan RM. Sjögren's syndrome: a review of aetiology, pathogenesis, diagnosis and management. Aust Dent J. 2010 Jun;55 Suppl 1:39-47. doi: 10.1111/j.1834-7819.2010.01197.x. Review. Citation on PubMed
- de Paiva CS, Pflugfelder SC. Mechanisms of Disease in Sjögren Syndrome-New Developments and Directions. Int J Mol Sci. 2020 Jan 19;21(2). pii: E650. doi: 10.3390/ijms21020650. Citation on PubMed
- de Paiva CS, Rocha EM. Sjögren syndrome: what and where are we looking for? Curr Opin Ophthalmol. 2015 Nov;26(6):517-25. doi: 10.1097/ICU.0000000000000208. Review. Citation on PubMed or Free article on PubMed Central
- Foulks GN, Forstot SL, Donshik PC, Forstot JZ, Goldstein MH, Lemp MA, Nelson JD, Nichols KK, Pflugfelder SC, Tanzer JM, Asbell P, Hammitt K, Jacobs DS. Clinical guidelines for management of dry eye associated with Sjögren disease. Ocul Surf. 2015 Apr;13(2):118-32. doi: 10.1016/j.jtos.2014.12.001. Epub 2015 Jan 15. Review. Citation on PubMed
- Jonsson R, Vogelsang P, Volchenkov R, Espinosa A, Wahren-Herlenius M, Appel S. The complexity of Sjögren's syndrome: novel aspects on pathogenesis. Immunol Lett. 2011 Dec 30;141(1):1-9. doi: 10.1016/j.imlet.2011.06.007. Epub 2011 Jul 12. Review. Citation on PubMed
- Jonsson R. Henrik Sjögren (1899-1986): the syndrome and his legacy. Ann Rheum Dis. 2021 Sep;80(9):1108-1109. doi: 10.1136/annrheumdis-2021-219942. Epub 2021 Jun 21. Citation on PubMed
- Mavragani CP, Moutsopoulos HM. Sjögren syndrome. CMAJ. 2014 Oct 21;186(15):E579-86. doi: 10.1503/cmaj.122037. Epub 2014 Feb 24. Review. Citation on PubMed or Free article on PubMed Central
- Peri Y, Agmon-Levin N, Theodor E, Shoenfeld Y. Sjögren's syndrome, the old and the new. Best Pract Res Clin Rheumatol. 2012 Feb;26(1):105-17. doi: 10.1016/j.berh.2012.01.012. Review. Citation on PubMed
- Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, Fisher BA, Gottenberg JE, Hernandez-Molina G, Kocher A, Kostov B, Kruize AA, Mandl T, Ng WF, Retamozo S, Seror R, Shoenfeld Y, Sisó-Almirall A, Tzioufas AG, Vitali C, Bowman S, Mariette X; EULAR-Sjögren Syndrome Task Force Group. EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies. Ann Rheum Dis. 2020 Jan;79(1):3-18. doi: 10.1136/annrheumdis-2019-216114. Epub 2019 Oct 31. Citation on PubMed