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Primary Sjögren syndrome

MedGen UID:
508757
•Concept ID:
C0151449
•
Disease or Syndrome
Synonyms: Primary Sjogren Syndrome; Primary Sjogren's syndrome; Primary Sjögren Syndrome; Primary Sjögren's syndrome
SNOMED CT: Primary Sjögren's syndrome (239912009); Primary Sjögren syndrome (239912009); Primary Sjogren's syndrome (239912009)

Definition

Sjogren syndrome without a concomitant systemic autoimmune disorder. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrimary Sjögren 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
An updated review of anti-Ro52 (TRIM21) antibodies impact in connective tissue diseases clinical management.
Decker P, Moulinet T, Pontille F, Cravat M, De Carvalho Bittencourt M, Jaussaud R
Autoimmun Rev 2022 Mar;21(3):103013. Epub 2021 Dec 9 doi: 10.1016/j.autrev.2021.103013. PMID: 34896652
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

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
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
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

Diagnosis

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
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
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

Therapy

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
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
Epithelial-immune cell interplay in primary Sjögren syndrome salivary gland pathogenesis.
Verstappen GM, Pringle S, Bootsma H, Kroese FGM
Nat Rev Rheumatol 2021 Jun;17(6):333-348. Epub 2021 Apr 28 doi: 10.1038/s41584-021-00605-2. PMID: 33911236Free 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
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

Prognosis

Artificial intelligence-driven drug development against autoimmune diseases.
Moingeon P
Trends Pharmacol Sci 2023 Jul;44(7):411-424. Epub 2023 May 31 doi: 10.1016/j.tips.2023.04.005. PMID: 37268540
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
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
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

Artificial intelligence-driven drug development against autoimmune diseases.
Moingeon P
Trends Pharmacol Sci 2023 Jul;44(7):411-424. Epub 2023 May 31 doi: 10.1016/j.tips.2023.04.005. PMID: 37268540
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
Cytokine profiles and clinical characteristics in primary Sjögren´s syndrome patient groups.
López-Villalobos EF, Muñoz-Valle JF, Palafox-Sánchez CA, García-Arellano S, Martínez-Fernández DE, Orozco-Barocio G, García-Espinoza JA, Oregon-Romero E
J Clin Lab Anal 2021 Feb;35(2):e23629. Epub 2020 Oct 17 doi: 10.1002/jcla.23629. PMID: 33070375Free PMC Article

Recent systematic reviews

TFOS Lifestyle Report: Impact of environmental conditions on the ocular surface.
Alves M, Asbell P, Dogru M, Giannaccare G, Grau A, Gregory D, Kim DH, Marini MC, Ngo W, Nowinska A, Saldanha IJ, Villani E, Wakamatsu TH, Yu M, Stapleton F
Ocul Surf 2023 Jul;29:1-52. Epub 2023 Apr 14 doi: 10.1016/j.jtos.2023.04.007. PMID: 37062427
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
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
Identification of lymphoma predictors in patients with primary Sjögren's syndrome: a systematic literature review and meta-analysis.
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. PMID: 24899571
Treatment of primary Sjögren syndrome: a systematic review.
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

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