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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

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
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

Characteristics and mortality in primary Sjögren syndrome-related interstitial lung disease.
Gao H, Sun Y, Zhang XY, Xie L, Zhang XW, Zhong YC, Zhang J, Hou YK, Li ZG
Medicine (Baltimore) 2021 Sep 3;100(35):e26777. doi: 10.1097/MD.0000000000026777. PMID: 34477117Free PMC Article
Prognostic markers of lymphoma development in primary Sjögren syndrome.
Retamozo S, Brito-Zerón P, Ramos-Casals M
Lupus 2019 Jul;28(8):923-936. Epub 2019 Jun 19 doi: 10.1177/0961203319857132. PMID: 31215845
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
Sjögren Syndrome and Pregnancy: A Literature Review.
Gupta S, Gupta N
Perm J 2017;21:16-047. Epub 2016 Nov 9 doi: 10.7812/TPP/16-047. PMID: 28080954Free 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

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
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
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
Primary Sjogren syndrome.
Ramos-Casals M, Brito-Zerón P, Sisó-Almirall A, Bosch X
BMJ 2012 Jun 14;344:e3821. doi: 10.1136/bmj.e3821. PMID: 22700787

Therapy

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
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

Characteristics and mortality in primary Sjögren syndrome-related interstitial lung disease.
Gao H, Sun Y, Zhang XY, Xie L, Zhang XW, Zhong YC, Zhang J, Hou YK, Li ZG
Medicine (Baltimore) 2021 Sep 3;100(35):e26777. doi: 10.1097/MD.0000000000026777. PMID: 34477117Free 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
Prognostic markers of lymphoma development in primary Sjögren syndrome.
Retamozo S, Brito-Zerón P, Ramos-Casals M
Lupus 2019 Jul;28(8):923-936. Epub 2019 Jun 19 doi: 10.1177/0961203319857132. PMID: 31215845
Sjögren Syndrome and Pregnancy: A Literature Review.
Gupta S, Gupta N
Perm J 2017;21:16-047. Epub 2016 Nov 9 doi: 10.7812/TPP/16-047. PMID: 28080954Free 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
Characteristics and mortality in primary Sjögren syndrome-related interstitial lung disease.
Gao H, Sun Y, Zhang XY, Xie L, Zhang XW, Zhong YC, Zhang J, Hou YK, Li ZG
Medicine (Baltimore) 2021 Sep 3;100(35):e26777. doi: 10.1097/MD.0000000000026777. PMID: 34477117Free PMC Article
Interleukin 6 receptor inhibition in primary Sjögren syndrome: a multicentre double-blind randomised placebo-controlled trial.
Felten R, Devauchelle-Pensec V, Seror R, Duffau P, Saadoun D, Hachulla E, Pierre Yves H, Salliot C, Perdriger A, Morel J, Mékinian A, Vittecoq O, Berthelot JM, Dernis E, Le Guern V, Dieudé P, Larroche C, Richez C, Martin T, Zarnitsky C, Blaison G, Kieffer P, Maurier F, Dellal A, Rist S, Andres E, Contis A, Chatelus E, Sordet C, Sibilia J, Arnold C, Tawk MY, Aberkane O, Holterbach L, Cacoub P, Saraux A, Mariette X, Meyer N, Gottenberg JE
Ann Rheum Dis 2021 Mar;80(3):329-338. Epub 2020 Nov 18 doi: 10.1136/annrheumdis-2020-218467. PMID: 33208345
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

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: 36086910
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
Pediatric primary Sjögren syndrome presenting with bilateral ranulas: A case report and systematic review of the literature.
Means C, Aldape MA, King E
Int J Pediatr Otorhinolaryngol 2017 Oct;101:11-19. Epub 2017 Jul 19 doi: 10.1016/j.ijporl.2017.07.019. PMID: 28964279
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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