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Sjögren syndrome

MedGen UID:
282890
Concept ID:
C1527336
Disease or Syndrome
Synonyms: SICCA SYNDROME; SJOGREN SYNDROME; Sjogren's Syndrome; Sjögren's syndrome
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: HPO
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in homozygotes. In the context of medical genetics, autosomal recessive disorders manifest in homozygotes (with two copies of the mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
SNOMED CT: Sjögren's disease (83901003); Sjögren's syndrome (83901003); Sjogren's syndrome (83901003); Sjögren syndrome (83901003); Sjogrens syndrome (83901003)
 
OMIM®: 270150

Definition

Sjogren syndrome is an autoimmune disease that mainly affects the exocrine glands. It is clinically characterized by keratoconjunctivitis sicca and xerostomia (Goransson et al., 2006). See 200400 for association of Sjogren syndrome with achalasia in sisters. [from OMIM]

Additional description

From GHR
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.When Sjögren syndrome first occurs on its own, it is called primary Sjögren syndrome. 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. In such cases, the individual is said to have secondary Sjögren syndrome. Other autoimmune disorders can also develop after the onset of primary Sjögren syndrome. In all, about half of all individuals with Sjögren syndrome also have another autoimmune disorder.  http://ghr.nlm.nih.gov/condition/sjogren-syndrome

Clinical features

Tear film insufficiency
MedGen UID:
4411
Concept ID:
C0013238
Disease or Syndrome
Dryness of the eye related to deficiency of the tear film components (aqueous, mucin, or lipid), lid surface abnormalities, or epithelial abnormalities. Keratoconjunctivitis sicca often results in a scratchy or sandy sensation (foreign body sensation) in the eyes, and may also be associated with itching, inability to produce tears, photosensitivity, redness, pain, and difficulty in moving the eyelids.
Autoimmune disease
MedGen UID:
2135
Concept ID:
C0004364
Disease or Syndrome
Your body's immune system protects you from disease and infection. But if you have an autoimmune disease, your immune system attacks healthy cells in your body by mistake. Autoimmune diseases can affect many parts of the body. No one is sure what causes autoimmune diseases. They do tend to run in families. Women - particularly African-American, Hispanic-American, and Native-American women - have a higher risk for some autoimmune diseases. There are more than 80 types of autoimmune diseases, and some have similar symptoms. This makes it hard for your health care provider to know if you really have one of these diseases, and if so, which one. Getting a diagnosis can be frustrating and stressful. Often, the first symptoms are fatigue, muscle aches and a low fever. The classic sign of an autoimmune disease is inflammation, which can cause redness, heat, pain and swelling. The diseases may also have flare-ups, when they get worse, and remissions, when symptoms get better or disappear. Treatment depends on the disease, but in most cases one important goal is to reduce inflammation. Sometimes doctors prescribe corticosteroids or other drugs that reduce your immune response.
Tear film insufficiency
MedGen UID:
4411
Concept ID:
C0013238
Disease or Syndrome
Dryness of the eye related to deficiency of the tear film components (aqueous, mucin, or lipid), lid surface abnormalities, or epithelial abnormalities. Keratoconjunctivitis sicca often results in a scratchy or sandy sensation (foreign body sensation) in the eyes, and may also be associated with itching, inability to produce tears, photosensitivity, redness, pain, and difficulty in moving the eyelids.
Abnormality of metabolism/homeostasis
MedGen UID:
867398
Concept ID:
C4021768
Finding
Rheumatoid arthritis
MedGen UID:
2078
Concept ID:
C0003873
Disease or Syndrome
Rheumatoid arthritis is an inflammatory disease, primarily of the joints, with autoimmune features and a complex genetic component.
Tear film insufficiency
MedGen UID:
4411
Concept ID:
C0013238
Disease or Syndrome
Dryness of the eye related to deficiency of the tear film components (aqueous, mucin, or lipid), lid surface abnormalities, or epithelial abnormalities. Keratoconjunctivitis sicca often results in a scratchy or sandy sensation (foreign body sensation) in the eyes, and may also be associated with itching, inability to produce tears, photosensitivity, redness, pain, and difficulty in moving the eyelids.
Xerostomia
MedGen UID:
22735
Concept ID:
C0043352
Disease or Syndrome
Dry mouth is the feeling that there is not enough saliva in your mouth. Everyone has a dry mouth once in a while - if they are nervous, upset or under stress. But if you have a dry mouth all or most of the time, it can be uncomfortable and can lead to serious health problems. Symptoms of dry mouth include. -A sticky, dry feeling in the mouth. -Trouble chewing, swallowing, tasting, or speaking. -A burning feeling in the mouth. -A dry feeling in the throat. -Cracked lips. -A dry, rough tongue. -Mouth sores. -An infection in the mouth. Dry mouth is not a normal part of aging. Causes include some medicines, radiation therapy, chemotherapy, and nerve damage. Salivary gland diseases, Sjogren's syndrome, HIV/AIDS, and diabetes can also cause dry mouth. Treatment depends on the cause. Things you can do include sipping water, avoiding drinks with caffeine, tobacco, and alcohol, and chewing sugarless gum or sucking on sugarless hard candy. NIH: National Institute of Dental and Craniofacial Research.

Term Hierarchy

Recent clinical studies

Etiology

Babazade R, Sun Z, Hesler BD, Sharma A, Makarova N, Dalton JE, Turan A
Anesth Analg 2015 Nov;121(5):1222-30. doi: 10.1213/ANE.0000000000000913. PMID: 26309019Free PMC Article
Priori R, Minniti A, Derme M, Antonazzo B, Brancatisano F, Ghirini S, Valesini G, Framarino-dei-Malatesta M
J Rheumatol 2015 Aug;42(8):1427-31. Epub 2015 Jul 1 doi: 10.3899/jrheum.141475. [Epub ahead of print] PMID: 26136488
Mathews PM, Hahn S, Hessen M, Kim J, Grader-Beck T, Birnbaum J, Baer AN, Akpek EK
Am J Ophthalmol 2015 Sep;160(3):447-452.e1. Epub 2015 Jun 17 doi: 10.1016/j.ajo.2015.06.004. [Epub ahead of print] PMID: 26093285
Gheitasi H, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Retamozo S, Ramos-Casals M, Brito-Zerón P; SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI)
Int Immunopharmacol 2015 Aug;27(2):194-9. Epub 2015 Apr 18 doi: 10.1016/j.intimp.2015.03.027. [Epub ahead of print] PMID: 25899085
Tzarouchi LC, Zikou AK, Tsifetaki N, Astrakas LG, Konitsiotis S, Voulgari P, Drosos A, Argyropoulou MI
AJNR Am J Neuroradiol 2014 Apr;35(4):680-5. Epub 2013 Nov 1 doi: 10.3174/ajnr.A3756. [Epub ahead of print] PMID: 24184520

Diagnosis

Babazade R, Sun Z, Hesler BD, Sharma A, Makarova N, Dalton JE, Turan A
Anesth Analg 2015 Nov;121(5):1222-30. doi: 10.1213/ANE.0000000000000913. PMID: 26309019Free PMC Article
Minato H, Kinoshita E, Nakada S, Nojima T, Tanaka M, Usuda K, Sagawa M, Iwao H, Tanaka M, Doai M, Takahashi T, Shibata N
Diagn Pathol 2015 Jul 16;10:103. doi: 10.1186/s13000-015-0332-y. [Epub ahead of print] PMID: 26173602Free PMC Article
Mathews PM, Hahn S, Hessen M, Kim J, Grader-Beck T, Birnbaum J, Baer AN, Akpek EK
Am J Ophthalmol 2015 Sep;160(3):447-452.e1. Epub 2015 Jun 17 doi: 10.1016/j.ajo.2015.06.004. [Epub ahead of print] PMID: 26093285
Gheitasi H, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Retamozo S, Ramos-Casals M, Brito-Zerón P; SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI)
Int Immunopharmacol 2015 Aug;27(2):194-9. Epub 2015 Apr 18 doi: 10.1016/j.intimp.2015.03.027. [Epub ahead of print] PMID: 25899085
Anand A, Krishna GG, Sibley RK, Kambham N
Am J Kidney Dis 2015 Sep;66(3):532-5. Epub 2015 Feb 4 doi: 10.1053/j.ajkd.2014.11.032. [Epub ahead of print] PMID: 25661680

Therapy

Kawakita T, Shimmura S, Tsubota K
Asia Pac J Ophthalmol (Phila) 2015 Mar-Apr;4(2):101-5. doi: 10.1097/APO.0000000000000040. PMID: 26065354
Gheitasi H, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Retamozo S, Ramos-Casals M, Brito-Zerón P; SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI)
Int Immunopharmacol 2015 Aug;27(2):194-9. Epub 2015 Apr 18 doi: 10.1016/j.intimp.2015.03.027. [Epub ahead of print] PMID: 25899085
Gottenberg JE, Ravaud P, Puéchal X, Le Guern V, Sibilia J, Goeb V, Larroche C, Dubost JJ, Rist S, Saraux A, Devauchelle-Pensec V, Morel J, Hayem G, Hatron P, Perdriger A, Sene D, Zarnitsky C, Batouche D, Furlan V, Benessiano J, Perrodeau E, Seror R, Mariette X
JAMA 2014 Jul 16;312(3):249-58. doi: 10.1001/jama.2014.7682. PMID: 25027140
Tzarouchi LC, Zikou AK, Tsifetaki N, Astrakas LG, Konitsiotis S, Voulgari P, Drosos A, Argyropoulou MI
AJNR Am J Neuroradiol 2014 Apr;35(4):680-5. Epub 2013 Nov 1 doi: 10.3174/ajnr.A3756. [Epub ahead of print] PMID: 24184520
Lieberman SM
Curr Opin Rheumatol 2013 Sep;25(5):651-7. doi: 10.1097/BOR.0b013e328363ed23. PMID: 23917159Free PMC Article

Prognosis

Brito-Zerón P, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Gheitasi H, Retamozo S, Ramos-Casals M; SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI)
Ann Rheum Dis 2016 Feb;75(2):348-55. Epub 2014 Nov 28 doi: 10.1136/annrheumdis-2014-206418. [Epub ahead of print] PMID: 25433020
Babazade R, Sun Z, Hesler BD, Sharma A, Makarova N, Dalton JE, Turan A
Anesth Analg 2015 Nov;121(5):1222-30. doi: 10.1213/ANE.0000000000000913. PMID: 26309019Free PMC Article
Shen CC, Yang AC, Kuo BI, Tsai SJ
J Rheumatol 2015 Jul;42(7):1203-8. Epub 2015 May 15 doi: 10.3899/jrheum.141361. [Epub ahead of print] PMID: 25979721
Kidder D, Rutherford E, Kipgen D, Fleming S, Geddes C, Stewart GA
Nephrol Dial Transplant 2015 Aug;30(8):1363-9. Epub 2015 Mar 27 doi: 10.1093/ndt/gfv042. [Epub ahead of print] PMID: 25817222
Lieberman SM
Curr Opin Rheumatol 2013 Sep;25(5):651-7. doi: 10.1097/BOR.0b013e328363ed23. PMID: 23917159Free PMC Article

Clinical prediction guides

Priori R, Minniti A, Derme M, Antonazzo B, Brancatisano F, Ghirini S, Valesini G, Framarino-dei-Malatesta M
J Rheumatol 2015 Aug;42(8):1427-31. Epub 2015 Jul 1 doi: 10.3899/jrheum.141475. [Epub ahead of print] PMID: 26136488
Gheitasi H, Kostov B, Solans R, Fraile G, Suárez-Cuervo C, Casanovas A, Rascón FJ, Qanneta R, Pérez-Alvarez R, Ripoll M, Akasbi M, Pinilla B, Bosch JA, Nava-Mateos J, Díaz-López B, Morera-Morales ML, Retamozo S, Ramos-Casals M, Brito-Zerón P; SS Study Group, Autoimmune Diseases Study Group (GEAS), Spanish Society of Internal Medicine (SEMI)
Int Immunopharmacol 2015 Aug;27(2):194-9. Epub 2015 Apr 18 doi: 10.1016/j.intimp.2015.03.027. [Epub ahead of print] PMID: 25899085
Anand A, Krishna GG, Sibley RK, Kambham N
Am J Kidney Dis 2015 Sep;66(3):532-5. Epub 2015 Feb 4 doi: 10.1053/j.ajkd.2014.11.032. [Epub ahead of print] PMID: 25661680
Tzarouchi LC, Zikou AK, Tsifetaki N, Astrakas LG, Konitsiotis S, Voulgari P, Drosos A, Argyropoulou MI
AJNR Am J Neuroradiol 2014 Apr;35(4):680-5. Epub 2013 Nov 1 doi: 10.3174/ajnr.A3756. [Epub ahead of print] PMID: 24184520
Krieger M, Roos A, Stendel C, Claeys KG, Sonmez FM, Baudis M, Bauer P, Bornemann A, de Goede C, Dufke A, Finkel RS, Goebel HH, Häussler M, Kingston H, Kirschner J, Medne L, Muschke P, Rivier F, Rudnik-Schöneborn S, Spengler S, Inzana F, Stanzial F, Benedicenti F, Synofzik M, Lia Taratuto A, Pirra L, Tay SK, Topaloglu H, Uyanik G, Wand D, Williams D, Zerres K, Weis J, Senderek J
Brain 2013 Dec;136(Pt 12):3634-44. Epub 2013 Oct 30 doi: 10.1093/brain/awt283. [Epub ahead of print] PMID: 24176978

Recent systematic reviews

Deng C, Hu C, Chen S, Li J, Wen X, Wu Z, Li Y, Zhang F, Li Y
PLoS One 2015;10(1):e0116744. Epub 2015 Jan 28 doi: 10.1371/journal.pone.0116744. PMID: 25629973Free PMC Article
Nishishinya MB, Pereda CA, Muñoz-Fernández S, Pego-Reigosa JM, Rúa-Figueroa I, Andreu JL, Fernández-Castro M, Rosas J, Loza Santamaría E
Rheumatol Int 2015 Jan;35(1):17-26. Epub 2014 Jun 5 doi: 10.1007/s00296-014-3051-x. [Epub ahead of print] PMID: 24899571
Yao Q, Altman RD, Wang X
J Clin Rheumatol 2012 Jan;18(1):28-32. doi: 10.1097/RHU.0b013e31823ecbdf. PMID: 22157271
Ramos-Casals M, Tzioufas AG, Stone JH, Sisó A, Bosch X
JAMA 2010 Jul 28;304(4):452-60. doi: 10.1001/jama.2010.1014. PMID: 20664046
Marsh P, Pflugfelder SC
Ophthalmology 1999 Apr;106(4):811-6. doi: 10.1016/S0161-6420(99)90171-9. PMID: 10201607

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