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Sjogren syndrome

MedGen UID:
282890
•Concept ID:
C1527336
•
Disease or Syndrome
Synonyms: SICCA SYNDROME; Sjogren's Syndrome; Sjögren's syndrome
SNOMED CT: Sjögren's disease (83901003); Sjögren's syndrome (83901003); Sjogren's syndrome (83901003); Sjögren syndrome (83901003); Sjogrens syndrome (83901003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0010030
OMIM®: 270150
Orphanet: ORPHA289390

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]

Clinical features

From HPO
Interstitial nephritis
MedGen UID:
11952
•Concept ID:
C0041349
•
Disease or Syndrome
A form of inflammation of the kidney affecting the interstitium of the kidneys surrounding the tubules.
See: Feature record | Search on this feature
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.
See: Feature record | Search on this feature
Autoimmunity
MedGen UID:
2136
•Concept ID:
C0004368
•
Pathologic Function
The occurrence of an immune reaction against the organism's own cells or tissues.
See: Feature record | Search on this feature
Keratoconjunctivitis sicca
MedGen UID:
9620
•Concept ID:
C0022575
•
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.
See: Feature record | Search on this feature
Abnormality of metabolism/homeostasis
MedGen UID:
867398
•Concept ID:
C4021768
•
Finding
See: Feature record | Search on this feature
Xerostomia
MedGen UID:
22735
•Concept ID:
C0043352
•
Disease or Syndrome
Dryness of the mouth due to salivary gland dysfunction.
See: Feature record | Search on this feature
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Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSjogren syndrome

Professional guidelines

PubMed

Update on the pathophysiology and treatment of primary Sjögren syndrome.
Baldini C, Fulvio G, La Rocca G, Ferro F
Nat Rev Rheumatol 2024 Aug;20(8):473-491. Epub 2024 Jul 9 doi: 10.1038/s41584-024-01135-3. PMID: 38982205
EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies.
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
Ann Rheum Dis 2020 Jan;79(1):3-18. Epub 2019 Oct 31 doi: 10.1136/annrheumdis-2019-216114. PMID: 31672775
Autoimmune Diseases and Their Manifestations on Oral Cavity: Diagnosis and Clinical Management.
Saccucci M, Di Carlo G, Bossù M, Giovarruscio F, Salucci A, Polimeni A
J Immunol Res 2018;2018:6061825. Epub 2018 May 27 doi: 10.1155/2018/6061825. PMID: 29977929Free PMC Article

Recent clinical studies

Etiology

Epidemiology of Sjögren syndrome.
Beydon M, McCoy S, Nguyen Y, Sumida T, Mariette X, Seror R
Nat Rev Rheumatol 2024 Mar;20(3):158-169. Epub 2023 Dec 18 doi: 10.1038/s41584-023-01057-6. PMID: 38110617
Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis.
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Autonomic neuropathies.
Kaur D, Tiwana H, Stino A, Sandroni P
Muscle Nerve 2021 Jan;63(1):10-21. Epub 2020 Sep 14 doi: 10.1002/mus.27048. PMID: 32926436
Lung complications of Sjogren syndrome.
Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Aug 18 doi: 10.1183/16000617.0021-2020. PMID: 32817113Free PMC Article
Sjögren syndrome.
Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M
Nat Rev Dis Primers 2016 Jul 7;2:16047. doi: 10.1038/nrdp.2016.47. PMID: 27383445

Diagnosis

Sjögren syndrome.
Brito-Zerón P, Retamozo S, Ramos-Casals M
Med Clin (Barc) 2023 Feb 24;160(4):163-171. Epub 2022 Dec 15 doi: 10.1016/j.medcli.2022.10.007. PMID: 36528400
Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis.
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Primary Sjögren's syndrome and the eye.
Bjordal O, Norheim KB, Rødahl E, Jonsson R, Omdal R
Surv Ophthalmol 2020 Mar-Apr;65(2):119-132. Epub 2019 Oct 18 doi: 10.1016/j.survophthal.2019.10.004. PMID: 31634487
Sjögren syndrome.
Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M
Nat Rev Dis Primers 2016 Jul 7;2:16047. doi: 10.1038/nrdp.2016.47. PMID: 27383445
Sensory neuronopathies.
Gwathmey KG
Muscle Nerve 2016 Jan;53(1):8-19. Epub 2015 Nov 23 doi: 10.1002/mus.24943. PMID: 26467754

Therapy

Current and future therapies for primary Sjögren syndrome.
Seror R, Nocturne G, Mariette X
Nat Rev Rheumatol 2021 Aug;17(8):475-486. Epub 2021 Jun 29 doi: 10.1038/s41584-021-00634-x. PMID: 34188206
Autonomic neuropathies.
Kaur D, Tiwana H, Stino A, Sandroni P
Muscle Nerve 2021 Jan;63(1):10-21. Epub 2020 Sep 14 doi: 10.1002/mus.27048. PMID: 32926436
Lung complications of Sjogren syndrome.
Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Aug 18 doi: 10.1183/16000617.0021-2020. PMID: 32817113Free PMC Article
EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies.
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
Ann Rheum Dis 2020 Jan;79(1):3-18. Epub 2019 Oct 31 doi: 10.1136/annrheumdis-2019-216114. PMID: 31672775
Lymphocytic Interstitial Pneumonia.
Panchabhai TS, Farver C, Highland KB
Clin Chest Med 2016 Sep;37(3):463-74. Epub 2016 Jun 25 doi: 10.1016/j.ccm.2016.04.009. PMID: 27514593

Prognosis

Sjögren syndrome.
Brito-Zerón P, Retamozo S, Ramos-Casals M
Med Clin (Barc) 2023 Feb 24;160(4):163-171. Epub 2022 Dec 15 doi: 10.1016/j.medcli.2022.10.007. PMID: 36528400
Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis.
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Autoimmune liver diseases in systemic rheumatic diseases.
Wang CR, Tsai HW
World J Gastroenterol 2022 Jun 21;28(23):2527-2545. doi: 10.3748/wjg.v28.i23.2527. PMID: 35949355Free PMC Article
Lung complications of Sjogren syndrome.
Luppi F, Sebastiani M, Sverzellati N, Cavazza A, Salvarani C, Manfredi A
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Aug 18 doi: 10.1183/16000617.0021-2020. PMID: 32817113Free PMC Article
Renal involvement in primary Sjögren syndrome.
François H, Mariette X
Nat Rev Nephrol 2016 Feb;12(2):82-93. Epub 2015 Nov 16 doi: 10.1038/nrneph.2015.174. PMID: 26568188

Clinical prediction guides

Genetics and epigenetics of primary Sjögren syndrome: implications for future therapies.
Thorlacius GE, Björk A, Wahren-Herlenius M
Nat Rev Rheumatol 2023 May;19(5):288-306. Epub 2023 Mar 13 doi: 10.1038/s41584-023-00932-6. PMID: 36914790Free PMC Article
Efficacy and Safety of Low-Dose Interleukin 2 for Primary Sjögren Syndrome: A Randomized Clinical Trial.
He J, Chen J, Miao M, Zhang R, Cheng G, Wang Y, Feng R, Huang B, Luan H, Jia Y, Jin Y, Zhang X, Shao M, Wang Y, Zhang X, Li J, Zhao X, Wang H, Liu T, Xiao X, Zhang X, Su Y, Mu R, Ye H, Li R, Liu X, Liu Y, Li C, Liu H, Hu F, Guo J, Liu W, Zhang WB, Jacob A, Ambrus JL Jr, Ding C, Yu D, Sun X, Li Z
JAMA Netw Open 2022 Nov 1;5(11):e2241451. doi: 10.1001/jamanetworkopen.2022.41451. PMID: 36355371Free PMC Article
Interstitial lung disease in Primary Sjögren's syndrome.
Lin W, Xin Z, Zhang J, Liu N, Ren X, Liu M, Su Y, Liu Y, Yang L, Guo S, Yang Y, Li Y, Cao J, Ning X, Li J, Xue H, Niu N, Chen Y, Li F, Sun L, Zhang X, Zhang F, Zhang W
BMC Pulm Med 2022 Feb 27;22(1):73. doi: 10.1186/s12890-022-01868-5. PMID: 35220958Free PMC Article
Sjögren syndrome and MIG.
Ruffilli I
Clin Ter 2019 Nov-Dec;170(6):e478-e482. doi: 10.7417/CT.2019.2179. PMID: 31696913
Sjögren syndrome.
Brito-Zerón P, Baldini C, Bootsma H, Bowman SJ, Jonsson R, Mariette X, Sivils K, Theander E, Tzioufas A, Ramos-Casals M
Nat Rev Dis Primers 2016 Jul 7;2:16047. doi: 10.1038/nrdp.2016.47. PMID: 27383445

Recent systematic reviews

Primary Sjögren syndrome-related peripheral neuropathy: A systematic review and meta-analysis.
Liampas A, Parperis K, Erotocritou MF, Nteveros A, Papadopoulou M, Moschovos C, Akil M, Coaccioli S, Hadjigeorgiou GM, Hadjivassiliou M, Zis P
Eur J Neurol 2023 Jan;30(1):255-265. Epub 2022 Sep 23 doi: 10.1111/ene.15555. PMID: 36086910Free PMC Article
Sjögren syndrome overlapping with ANCA-associated vasculitis: Four additional cases and systematic literature review.
Coustal C, Guillope B, Serrand C, Morel J, Taieb G, Castille E, Meliani K, Darmon O, Goulabchand R, Guilpain P
Autoimmun Rev 2022 Jun;21(6):103099. Epub 2022 Apr 19 doi: 10.1016/j.autrev.2022.103099. PMID: 35452855
Ocular Manifestations in Rheumatoid Arthritis, Connective Tissue Disease, and Vasculitis: A Systematic Review and Metaanalysis.
Turk MA, Hayworth JL, Nevskaya T, Pope JE
J Rheumatol 2021 Jan 1;48(1):25-34. Epub 2020 May 1 doi: 10.3899/jrheum.190768. PMID: 32358156
Efficacy and safety of topical and systemic medications: a systematic literature review informing the EULAR recommendations for the management of Sjögren's syndrome.
Brito-Zerón P, Retamozo S, Kostov B, Baldini C, Bootsma H, De Vita S, Dörner T, Gottenberg JE, Kruize AA, Mandl T, Ng WF, Seror R, Tzioufas AG, Vitali C, Bowman S, Mariette X, Ramos-Casals M
RMD Open 2019;5(2):e001064. Epub 2019 Oct 28 doi: 10.1136/rmdopen-2019-001064. PMID: 31749986Free PMC Article
Characterization of systemic disease in primary Sjögren's syndrome: EULAR-SS Task Force recommendations for articular, cutaneous, pulmonary and renal involvements.
Ramos-Casals M, Brito-Zerón P, Seror R, Bootsma H, Bowman SJ, Dörner T, Gottenberg JE, Mariette X, Theander E, Bombardieri S, De Vita S, Mandl T, Ng WF, Kruize A, Tzioufas A, Vitali C; EULAR Sjögren Syndrome Task Force
Rheumatology (Oxford) 2015 Dec;54(12):2230-8. Epub 2015 Jul 31 doi: 10.1093/rheumatology/kev200. PMID: 26231345Free PMC Article

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