Skip to main content
SpringerLink
Log in

Epidemiology

  • Chapter
  • First Online:
Sjögren’s Syndrome and the Salivary Glands

Part of the book series: Contemporary Rheumatology ((CR))

  • 539 Accesses

Abstract

The reported incidence and prevalence of primary Sjögen’s Syndrome in the literature vary greatly depending on the applied classification criteria. Furthermore, factors such as age, gender, geographic, and ethnicity-specific differences greatly impact the reported incidence and prevalence including the clinical presentation and mortality.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 99.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Manthorpe R, Oxholm P, Prause JU, Schiødt M. The Copenhagen criteria for Sjögren’s syndrome. Scand J Rheumatol Suppl. 1986;61:19–21.

    CAS  PubMed  Google Scholar 

  2. Vitali C, Bombardieri S, Moutsopoulos HM, Balestrieri G, Bencivelli W, Bernstein RM, et al. Preliminary classification criteria for Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum. 1993;36:340–7.

    CAS  PubMed  Google Scholar 

  3. Vitali C, Bombardieri S, Jonsson R, Moutsopoulos HM, Alexander EL, Carsons SE, Daniels TE, Fox PC, Fox RI, Kassan SS, Pillemer SR, Talal N, Weisman MH, European Study Group on Classification Criteria for Sjögren’s syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61(6):554–8.

    CAS  PubMed  PubMed Central  Google Scholar 

  4. Shiboski CH, Shiboski SC, Seror R, Criswell LA, Labetoulle M, Lietman TM, Rasmussen A, Scofield H, Vitali C, Bowman SJ, Mariette X, International Sjögren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European league against rheumatism classification criteria for primary Sjogren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Arthritis Rheumatol. 2017;69(1):35–45.

    PubMed  Google Scholar 

  5. Binard A, Devauchelle-Pensec V, Fautrel B, Jousse S, Youinou P, Saraux A. Epidemiology of Sjögren’s syndrome: where are we now? Clin Exp Rheumatol. 2007;25:1–4. 5.

    CAS  PubMed  Google Scholar 

  6. Thomas E, Hay EM, Hajeer A, Silman J. Sjögren’s syndrome: a community-based study of prevalence and impact. Br J Rheumatol. 1998;37:1069–76. 6.

    CAS  PubMed  Google Scholar 

  7. Bowman SJ, Ibrahim GH, Holmes G, Hamburger J, Ainsworth JR. Estimating the prevalence among Caucasian women of primary Sjogren’s syndrome in two general practices in Birmingham, UK. Scand J Rheumatol. 2004;33:39–43. 8.

    CAS  PubMed  Google Scholar 

  8. Haugen AJ, Peen E, Hultén B, Johannessen AC, Brun JG, Halse AK, et al. Estimation of the prevalence of primary Sjögren’s syndrome in two age-different community-based populations using two sets of classification criteria: the Hordaland health study. Scand J Rheumatol. 2008;37:30–4.

    CAS  PubMed  Google Scholar 

  9. Qin B, Wang J, Yang Z, Yang M, Ma N, Huang F, Zhong R. Epidemiology of primary Sjögren’s syndrome: a systematic review and meta-analysis. Ann Rheum Dis. 2015;74(11):1983–9.

    CAS  PubMed  Google Scholar 

  10. Brito-Zerón P, Acar-Denizli N, Zeher M, Rasmussen A, Seror R, Theander E, Li X, Baldini C, Gottenberg JE, Danda D, Quartuccio L, Priori R, Hernandez-Molina G, Kruize AA, Valim V, Kvarnstrom M, Sene D, Gerli R, Praprotnik S, Isenberg D, Solans R, Rischmueller M, Kwok SK, Nordmark G, Suzuki Y, Giacomelli R, Devauchelle-Pensec V, Bombardieri M, Hofauer B, Bootsma H, Brun JG, Fraile G, Carsons SE, Gheita TA, Morel J, Vollenveider C, Atzeni F, Retamozo S, Horvath IF, Sivils K, Mandl T, Sandhya P, De Vita S, Sanchez-Guerrero J, van der Heijden E, Trevisani VFM, Wahren-Herlenius M, Mariette X, Ramos-Casals M, EULAR-SS Task Force Big Data Consortium. Influence of geolocation and ethnicity on the phenotypic expression of primary Sjogren’s syndrome at diagnosis in 8310 patients: a cross-sectional study from the Big Data Sjogren Project Consortium. Ann Rheum Dis. 2017;76(6):1042–50.

    PubMed  Google Scholar 

  11. Acar-Denizli N, Kostov B, Ramos-Casals M, Sjögren Big Data Consortium. The big data Sjögren Consortium: a project for a new data science era. Clin Exp Rheumatol. 2019;37 Suppl 118(3):19–23.

    PubMed  Google Scholar 

  12. Suri V, Suri V. Menopause and oral health. J Life Health. 2014;5(3):115–20.

    Google Scholar 

  13. Schaumberg DA, Sullivan DA, Buring JE, Dana MR. Prevalence of dry eye syndrome among US women. Am J Ophthalmol. 2003;136(2):318–26.

    PubMed  Google Scholar 

  14. Ramos-Casals M, Brito-Zerón P, Kostov B, Sisó-Almirall A, Bosch X, Buss D, Trilla A, Stone JH, Khamashta MA, Shoenfeld Y. Google-driven search for big data in autoimmune geoepidemiology: analysis of 394,827 patients with systemic autoimmune diseases. Autoimmun Rev. 2015;14(8):670–9.

    PubMed  Google Scholar 

  15. Li X, Xu B, Ma Y, Li X, Cheng Q, Wang X, Wang G, Qian L, Wei L. Clinical and laboratory profiles of primary Sjogren’s syndrome in a Chinese population: a retrospective analysis of 315 patients. Int J Rheum Dis. 2015;18(4):439–46.

    CAS  PubMed  Google Scholar 

  16. Ramos-Casals M, Acar-Denizli N, Vissink A, Brito-Zerón P, Li X, Carubbi F, Priori R, Toplak N, Baldini C, Faugier-Fuentes E, Kruize AA, Mandl T, Tomiita M, Gandolfo S, Hashimoto K, Hernandez-Molina G, Hofauer B, Mendieta-Zerón S, Rasmussen A, Sandhya P, Sene D, Trevisani VFM, Isenberg D, Sundberg E, Pasoto SG, Sebastian A, Suzuki Y, Retamozo S, Xu B, Giacomelli R, Gattamelata A, Bizjak M, Bombardieri S, Loor-Chavez RE, Hinrichs A, Olsson P, Bootsma H, Lieberman SM, Sjogren Big Data Consortium. Childhood-onset of primary Sjogren’s syndrome: phenotypic characterization at diagnosis of 158 children. Rheumatology (Oxford). 2021:keab032.

    Google Scholar 

  17. Virdee S, Greenan-Barrett J, Ciurtin C. A systematic review of primary Sjögren’s syndrome in male and paediatric populations. Clin Rheumatol. 2017;36(10):2225–36.

    PubMed  PubMed Central  Google Scholar 

  18. Sandhya P, Jeyaseelan L, Scofield RH, Danda D. Clinical characteristics and outcome of primary Sjogren’s syndrome: a large Asian Indian cohort. Open Rheumatol J. 2015;9:36–45.

    CAS  PubMed  PubMed Central  Google Scholar 

  19. Birlik M, Akar S, Gurler O, Sari I, Birlik B, Sarioglu S, Oktem MA, Saglam F, Can G, Kayahan H, Akkoc N, Onen F. Prevalence of primary Sjogren’s syndrome in Turkey: a population-based epidemiological study. Int J Clin Pract. 2009;63(6):954–61.

    CAS  PubMed  Google Scholar 

  20. Lyne SA, Downie-Doyle S, Lester SE, Quinlivan A, Toby Coates P, Gordon TP, Rischmueller M. Primary Sjögren’s syndrome in South Australia. Clin Exp Rheumatol. 2020;38 Suppl 126(4):57–63.

    PubMed  Google Scholar 

  21. Maciel G, Crowson CS, Matteson EL, Cornec D. Prevalence of primary Sjogren’s syndrome in a US population-based cohort. Arthritis Care Res (Hoboken). 2017;69(10):1612–6.

    Google Scholar 

  22. Izmirly PM, Buyon JP, Wan I, Belmont HM, Sahl S, Salmon JE, Askanase A, Bathon JM, Geraldino-Pardilla L, Ali Y, Ginzler EM, Putterman C, Gordon C, Helmick CG, Parton H. The incidence and prevalence of adult primary Sjögren’s syndrome in New York County. Arthritis Care Res (Hoboken). 2019;71(7):949–60.

    Google Scholar 

  23. Maldini C, Seror R, Fain O, Dhote R, Amoura Z, De Bandt M, Delassus JL, Falgarone G, Guillevin L, Le Guern V, Lhote F, Meyer O, Ramanoelina J, Sacré K, Uzunhan Y, Leroux JL, Mariette X, Mahr A. Epidemiology of primary Sjögren’s syndrome in a French multiracial/multiethnic area. Arthritis Care Res (Hoboken). 2014;66(3):454–63.

    Google Scholar 

  24. Narváez J, Sánchez-Fernández SÁ, Seoane-Mato D, Díaz-González F, Bustabad S. Prevalence of Sjögren’s syndrome in the general adult population in Spain: estimating the proportion of undiagnosed cases. Sci Rep. 2020;10(1):10627.

    PubMed  PubMed Central  Google Scholar 

  25. Gøransson LG, Haldorsen K, Brun JG, Harboe E, Jonsson MV, Skarstein K, Time K, Omdal R. The point prevalence of clinically relevant primary Sjogren’s syndrome in two Norwegian counties. Scand J Rheumatol. 2011;40(3):221–4.

    PubMed  Google Scholar 

  26. Lin D-F, Yan S-M, Zhao Y, et al. Clinical and prognostic characteristics of 573 cases of primary Sjogren’s syndrome. Chin Med J. 2010;123:3252–7.

    PubMed  Google Scholar 

  27. Alamanos Y, Tsifetaki N, Voulgari PV, Venetsanopoulou AI, Siozos C, Drosos AA. Epidemiology of primary Sjögren’s syndrome in north-West Greece, 1982-2003. Rheumatology (Oxford). 2006;45(2):187–91.

    CAS  Google Scholar 

  28. Plesivcnik Novljan M, Rozman B, Hocevar A, Grmek M, Kveder T, Tomsic M. Incidence of primary Sjogren’s syndrome in Slovenia. Ann Rheum Dis. 2004;63(7):874–6.

    CAS  PubMed  Google Scholar 

  29. Botsios C, Furlan A, Ostuni P, Sfriso P, Andretta M, Ometto F, Raffeiner B, Todesco S, Punzi L. Elderly onset of primary Sjögren’s syndrome: clinical manifestations, serological features and oral/ocular diagnostic tests. Comparison with adult and young onset of the disease in a cohort of 336 Italian patients. Joint Bone Spine. 2011;78(2):171–4.

    PubMed  Google Scholar 

  30. García-Carrasco M, Cervera R, Rosas J, Ramos-Casals M, Morlà RM, Sisó A, Jiménez S, Pallarés L, Font J, Ingelmo M. Primary Sjögren’s syndrome in the elderly: clinical and immunological characteristics. Lupus. 1999;8(1):20–3.

    PubMed  Google Scholar 

  31. Zhang NZ, Shi CS, Yao QP, Pan GX, Wang LL, Wen ZX, Li XC, Dong Y. Prevalence of primary Sjogren’s syndrome in China. J Rheumatol. 1995;22(4):659–61.

    CAS  PubMed  Google Scholar 

  32. Eaton WW, Rose NR, Kalaydjian A, Pedersen MG, Mortensen PB. Epidemiology of autoimmune diseases in Denmark. J Autoimmun. 2007;29(1):1–9.

    PubMed  PubMed Central  Google Scholar 

  33. Singh AG, Singh S, Matteson EL. Rate, risk factors and causes of mortality in patients with Sjögren’s syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford). 2016;55(3):450–60.

    Google Scholar 

  34. Sardu C, Cocco E, Mereu A, Massa R, Cuccu A, Marrosu MG, Contu P. Population based study of 12 autoimmune diseases in Sardinia, Italy: prevalence and comorbidity. PLoS One. 2012;7(3):e32487.

    CAS  PubMed  PubMed Central  Google Scholar 

  35. See L-C, Kuo C-F, Chou I-J, Chiou M-J, Yu K-H. Sex- and age-specific incidence of autoimmune rheumatic diseases in the Chinese population: a Taiwan population-based study. Semin Arthritis Rheum. 2013;43:381–6.

    PubMed  Google Scholar 

  36. Pertovaara M, Pukkala E, Laippala P, Miettinen A, Pasternack A. A longitudinal cohort study of Finnish patients with primary Sjögren’s syndrome: clinical, immunological, and epidemiological aspects. Ann Rheum Dis. 2001;60(5):467–72.

    CAS  PubMed  PubMed Central  Google Scholar 

  37. Tolentino Júnior DS, de Oliveira CM, de Assis EM. Population-based study of 24 autoimmune diseases carried out in a Brazilian microregion. J Epidemiol Glob Health. 2019;9(4):243–51.

    PubMed  PubMed Central  Google Scholar 

  38. Weng M-Y, Huang Y-T, Liu M-F, Lu T-H. Incidence and mortality of treated primary Sjogren’s syndrome in Taiwan: a population-based study. J Rheumatol. 2011;38:706–8.

    PubMed  Google Scholar 

  39. Yu K-H, See L-C, Kuo C-F, Chou I-J, Chou M-J. Prevalence and incidence in patients with autoimmune rheumatic diseases: a nationwide population-based study in Taiwan. Arthritis Care Res. 2013;65:244–50.

    Google Scholar 

  40. Alani H, Henty JR, Thompson NL, Jury E, Ciurtin C. Systematic review and meta-analysis of the epidemiology of polyautoimmunity in Sjögren’s syndrome (secondary Sjögren’s syndrome) focusing on autoimmune rheumatic diseases. Scand J Rheumatol. 2018;47(2):141–54.

    CAS  PubMed  Google Scholar 

  41. Moerman RV, Arends S, Mossel E, Kroese FGM, Vissink A, Bootsma H. 10-year follow-up of patients with rheumatoid arthritis and secondary Sjögren’s syndrome or sicca symptoms in daily clinical practice. Clin Exp Rheumatol. 2020;38 Suppl 126(4):64–72.

    PubMed  Google Scholar 

  42. Baer AN, Maynard JW, Shaikh F, Magder LS, Petri M. Secondary Sjogren’s syndrome in systemic lupus erythematosus defines a distinct disease subset. J Rheumatol. 2010;37(6):1143–9.

    PubMed  Google Scholar 

  43. Baldini C, Mosca M, Della Rossa A, Pepe P, Notarstefano C, Ferro F, et al. Overlap of ACA-positive systemic sclerosis and Sjogren’s syndrome: a distinct clinical entity with mild organ involvement but at high risk of lymphoma. Clin Exp Rheumatol. 2013;31:272–80.

    PubMed  Google Scholar 

  44. Martens PB, Pillemer SR, Jacobsson LT, O’Fallon WM, Matteson EL. Survivorship in a population based cohort of patients with Sjögren’s syndrome, 1976-1992. J Rheumatol. 1999;26(6):1296–300.

    CAS  PubMed  Google Scholar 

  45. Liang Y, Yang Z, Qin B, Zhong R. Primary Sjogren’s syndrome and malignancy risk: a systematic review and meta-analysis. Ann Rheum Dis. 2014;73(6):1151–6. https://doi.org/10.1136/annrheumdis-2013-203305.

    Article  PubMed  Google Scholar 

  46. Goules AV, Argyropoulou OD, Pezoulas VC, Chatzis L, Critselis E, Gandolfo S, Ferro F, Binutti M, Donati V, Zandonella Callegher S, Venetsanopoulou A, Zampeli E, Mavrommati M, Voulgari PV, Exarchos T, Mavragani CP, Baldini C, Skopouli FN, Fotiadis DI, De Vita S, Moutsopoulos HM, Tzioufas AG. Primary Sjögren’s syndrome of early and late onset: distinct clinical phenotypes and lymphoma development. Front Immunol. 2020;11:594096.

    CAS  PubMed  PubMed Central  Google Scholar 

  47. Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum. 2002;46(3):741–7.

    PubMed  Google Scholar 

  48. Reveille JD, Wilson RW, Provost TT, Bias WB, Arnett FC. Primary Sjögren’s syndrome and other autoimmune diseases in families. Prevalence and immunogenetic studies in six kindreds. Ann Intern Med. 1984;101(6):748–56.

    CAS  PubMed  Google Scholar 

  49. Kuo CF, Grainge MJ, Valdes AM, See LC, Luo SF, Yu KH, Zhang W, Doherty M. Familial risk of Sjogren’s syndrome and co-aggregation of autoimmune diseases in affected families: a nationwide population study. Arthritis Rheumatol. 2015;67(7):1904–12.

    PubMed  PubMed Central  Google Scholar 

  50. Lessard CJ, et al. Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren’s syndrome. Nat Genet. 2013;45:1284–92.

    CAS  PubMed  Google Scholar 

  51. Li Y, et al. A genome-wide association study in Han Chinese identifies a susceptibility locus for primary Sjögren’s syndrome at 7q11.23. Nat Genet. 2013;45:1361–5.

    CAS  PubMed  Google Scholar 

  52. Gottenberg J-E, et al. In primary Sjögren’s syndrome, HLA class II is associated exclusively with autoantibody production and spreading of the autoimmune response. Arthritis Rheumatol. 2003;48:2240–5.

    CAS  Google Scholar 

  53. Cruz-Tapias P, Rojas-Villarraga A, Maier-Moore S, Anaya JM. HLA and Sjögren’s syndrome susceptibility. A meta-analysis of worldwide studies. Autoimmun Rev. 2012;11(4):281–7.

    CAS  PubMed  Google Scholar 

  54. Kang HI, Fei HM, Saito I, Sawada S, Chen SL, Yi D, Chan E, Peebles C, Bugawan TL, Erlich HA, et al. Comparison of HLA class II genes in Caucasoid, Chinese, and Japanese patients with primary Sjögren’s syndrome. J Immunol. 1993;150(8 Pt 1):3615–23.

    CAS  PubMed  Google Scholar 

  55. Li Y, Zhang K, Chen H, Sun F, Xu J, Wu Z, Li P, Zhang L, Du Y, Luan H, Li X, Wu L, Li H, Wu H, Li X, Li X, Zhang X, Gong L, Dai L, Sun L, Zuo X, Xu J, Gong H, Li Z, Tong S, Wu M, Li X, Xiao W, Wang G, Zhu P, Shen M, Liu S, Zhao D, Liu W, Wang Y, Huang C, Jiang Q, Liu G, Liu B, Hu S, Zhang W, Zhang Z, You X, Li M, Hao W, Zhao C, Leng X, Bi L, Wang Y, Zhang F, Shi Q, Qi W, Zhang X, Jia Y, Su J, Li Q, Hou Y, Wu Q, Xu D, Zheng W, Zhang M, Wang Q, Fei Y, Zhang X, Li J, Jiang Y, Tian X, Zhao L, Wang L, Zhou B, Li Y, Zhao Y, Zeng X, Ott J, Wang J, Zhang F. A genome-wide association study in Han Chinese identifies a susceptibility locus for primary Sjögren’s syndrome at 7q11.23. Nat Genet. 2013;45(11):1361–5. https://doi.org/10.1038/ng.2779. Epub 2013 Oct 6.

    Article  CAS  PubMed  Google Scholar 

  56. Fox RI, et al. Primary Sjögren syndrome: clinical and immunological features. Semin Arthritis Rheum. 1984;14(2):77–105.

    CAS  PubMed  Google Scholar 

  57. van Woerkom JM, Kruize AA, Wenting-van Wijk MJ, Knol E, Bihari IC, Jacobs JW, Bijlsma JW, Lafeber FP, van Roon JA. Salivary gland and peripheral blood T helper 1 and 2 cell activity in Sjögren’s syndrome compared with non-Sjögren’s sicca syndrome. Ann Rheum Dis. 2005;64(10):1474–9.

    PubMed  PubMed Central  Google Scholar 

  58. Szabo K, Papp G, Barath S, Gyimesi E, Szanto A, Zeher M. Follicular helper T cells may play an important role in the severity of primary Sjögren’s syndrome. Clin Immunol. 2013;147(2):95–104.

    CAS  PubMed  Google Scholar 

  59. Lessard CJ, Li H, Adrianto I, Ice JA, Rasmussen A, Grundahl KM, Kelly JA, Dozmorov MG, Miceli-Richard C, Bowman S, Lester S, Eriksson P, Eloranta ML, Brun JG, Gøransson LG, Harboe E, Guthridge JM, Kaufman KM, Kvarnström M, Jazebi H, Cunninghame Graham DS, Grandits ME, Nazmul-Hossain AN, Patel K, Adler AJ, Maier-Moore JS, Farris AD, Brennan MT, Lessard JA, Chodosh J, Gopalakrishnan R, Hefner KS, Houston GD, Huang AJ, Hughes PJ, Lewis DM, Radfar L, Rohrer MD, Stone DU, Wren JD, Vyse TJ, Gaffney PM, James JA, Omdal R, Wahren-Herlenius M, Illei GG, Witte T, Jonsson R, Rischmueller M, Rönnblom L, Nordmark G, Ng WF, UK Primary Sjögren’s Syndrome Registry, Mariette X, Anaya JM, Rhodus NL, Segal BM, Scofield RH, Montgomery CG, Harley JB, Sivils KL. Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjogren’s syndrome. Nat Genet. 2013;45(11):1284–92.

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Center for Rheumatology and Spine Diseases, Copenhagen Center for Arthritis Research, Rigshospitalet, Copenhagen, Denmark

    Viktoria Fana & Lene Terslev

  2. Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark

    Lene Terslev

Authors
  1. Viktoria Fana
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lene Terslev
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Viktoria Fana .

Editor information

Editors and Affiliations

  1. Reumakliniek Lelystad, Lelystad, The Netherlands

    George A. W. Bruyn

Rights and permissions

Reprints and permissions

Copyright information

© 2022 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Fana, V., Terslev, L. (2022). Epidemiology. In: Bruyn, G.A.W. (eds) Sjögren’s Syndrome and the Salivary Glands. Contemporary Rheumatology. Springer, Cham. https://doi.org/10.1007/978-3-030-90977-2_2

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-90977-2_2

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-90976-5

  • Online ISBN: 978-3-030-90977-2

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation