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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
Tubulointerstitial 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

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
Reviewing primary Sjögren's syndrome: beyond the dryness - From pathophysiology to diagnosis and treatment.
Both T, Dalm VA, van Hagen PM, van Daele PL
Int J Med Sci 2017;14(3):191-200. Epub 2017 Feb 23 doi: 10.7150/ijms.17718. PMID: 28367079Free PMC Article

Recent clinical studies

Etiology

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
Rheumatologic Disorders and the Nervous System.
Pavlakis PP
Continuum (Minneap Minn) 2020 Jun;26(3):591-610. doi: 10.1212/CON.0000000000000856. PMID: 32487898
Osteoporosis in Rheumatic Diseases.
Adami G, Fassio A, Rossini M, Caimmi C, Giollo A, Orsolini G, Viapiana O, Gatti D
Int J Mol Sci 2019 Nov 22;20(23) doi: 10.3390/ijms20235867. PMID: 31766755Free 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
Neurologic Manifestations of Primary Sjögren Syndrome.
Margaretten M
Rheum Dis Clin North Am 2017 Nov;43(4):519-529. Epub 2017 Aug 23 doi: 10.1016/j.rdc.2017.06.002. PMID: 29061239
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

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
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
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
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

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
An Overview of Neonatal Lupus with Anti-Ro Characteristics.
Gryka-Marton M, Szukiewicz D, Teliga-Czajkowska J, Olesinska M
Int J Mol Sci 2021 Aug 27;22(17) doi: 10.3390/ijms22179281. PMID: 34502221Free PMC Article
B cells in the pathogenesis of primary Sjögren syndrome.
Nocturne G, Mariette X
Nat Rev Rheumatol 2018 Mar;14(3):133-145. Epub 2018 Feb 8 doi: 10.1038/nrrheum.2018.1. PMID: 29416129
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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