Skip to main content
SpringerLink
Log in

Epidemiologie des primären Sjögren-Syndroms

Epidemiology of primary Sjörgren’s syndrome

  • Leitthema
  • Published:
Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Das nach den AECG-Kriterien diagnostizierte primäre Sjögren-Syndrom (pSS) ist mit einer Prävalenz von rund 0,2% in der erwachsenen Normalbevölkerung und einer Jahresinzidenz von 4/100.000 Gesamtbevölkerung deutlich seltener als bisher angenommen. Auch der wiederkehrend berichtete Anteil von einem Mann auf 9 Frauen dürfte eher bei etwa 1:20 liegen. An pSS erkrankte Männer haben seltener immunologische, histopathologische oder sialographische Befunde und Organbeteiligungen.

Das Erkrankungsalter liegt in jüngeren Untersuchungen mit rund 45 Jahren um rund 10 Jahre niedriger als in solchen des letzten Jahrzehnts. Im jüngeren Alter erkrankte Patienten sind häufiger Rheumafaktor- und/oder Anti-Ro/SS-A-positiv und leiden häufiger an Hypokomplementämie oder Lymphadenopathien. Mit der Einführung der spezifischeren AECG-Kriterien finden sich weniger Männer, mehr jüngere und mehr schwer erkrankte Patienten in pSS-Kohorten. Etwa 5–10% der pSS-Patienten spezialisierter Zentren leiden an schwerwiegenden extraglandulären Manifestationen. Diese zeigen sich zumeist früh im Krankheitsverlauf und gehen z. T. mit erhöhter Mortalität einher. Betroffene ohne früh auftretende Organbeteiligungen können mehrheitlich mit einem leichten bis mäßig schweren Verlauf rechnen.

In Deutschland wird nur rund ein Zehntel der Sjögren-Kranken rheumatologisch betreut. Zusammen mit den nach AECG-Kriterien nicht berücksichtigten Anti-Ro/La-negativen Sjögren-Kranken und den Patienten mit sekundärem Sjögren-Syndrom dürften mindestens 0,4% der erwachsenen Bevölkerung unter stark beeinträchtigender immunpathologischer Sicca-Symptomatik leiden.

Abstract

According to the classification criteria of the American-European Consensus Group (AECG), the prevalence of primary Sjögren’s syndrome (pSS) of about 0.2% in the adult population and a yearly incidence of 4/100.000 in the general population are far lower than previously assumed. Moreover, the repeatedly reported male/female ratio of 1:9 seems to lie more in the range of 1:20. Male pSS patients show fewer immunological, histopathological or sialographic findings and organ involvement. Information on age at disease onset has also changed over the last decade. Recent studies indicate an onset age of approximately 45 years as compared to 56 in earlier studies of the last decade. Patients with an early disease onset are more frequently positive for rheumatoid factor (RF) and/or anti-Ro/SS-A. These patients also seem to have a higher risk of developing hypocomplementemia or lymphadenopathy.

As compared to earlier cohorts, the introduction of the rather specific AECG criteria will probably result in the participation of fewer men, younger patients in general and of more seriously ill patients in future cohorts. The change in the spectrum of pSS patients obviously reflects the altered classification criteria since the AECG criteria require anti-Ro/La positivity and therefore exclude a high number of patients with other immunological markers who also show severe sicca symptoms and organ involvements. About 5%–10% of pSS patients in rheumatological care suffer from severe extraglandular manifestations, which generally occur soon after disease onset. In particular, palpable purpura, hypocomplementemia, cryoglobulinemia and lymphoma are associated with increased mortality.

In Germany, approximately one tenth of Sjögren syndrome patients receive specialized rheumatological care. There is still insufficient knowledge about the vast majority of pSS patients who are not treated by rheumatologists. These patients, as well as all those who, according to the AECG criteria, are not classified as having pSS either due to anti-Ro/La negativity or having secondary Sjögren’s syndrome, probably add up to at least 0.4% of the adult population which, at present, suffers from considerable immunopathologic sicca symptoms.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Literatur

  1. Kassan SS, Moutsopoulos HM (2004) Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med 164(12):1275–1284

    Article  PubMed  Google Scholar 

  2. Birlik M, Akar S, Gurler O et al (2009) Prevalence of primary Sjögren’s syndrome in Turkey: a population-based epidemiological study. Int J Clin Pract 63(6):954–961

    Article  CAS  PubMed  Google Scholar 

  3. Vitali C, Bombardieri S, Jonsson R et al (2002) Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61(6):554–558

    Article  CAS  PubMed  Google Scholar 

  4. Manthorpe R, Jacobsson LT, Kirtava Z, Theander E (1998) Epidemiology of Sjögren’s syndrome, especially its primary form. Ann Med Interne (Paris) 149(1):7–11

    Google Scholar 

  5. Andrianakos A, Trontzas P, Christoyannis F et al (2003) Prevalence of rheumatic diseases in Greece: a cross-sectional population based epidemiological study. The ESORDIG Study. J Rheumatol 30(7):1589–1601

    PubMed  Google Scholar 

  6. Bjerrum KB (1997) Keratoconjunctivitis sicca and primary Sjögren’s syndrome in a Danish population aged 30–60 years. Acta Ophthalmol Scand 75(3):281–286

    Article  CAS  PubMed  Google Scholar 

  7. Thomas E, Hay EM, Hajeer A, Silman AJ (1998) Sjögren’s syndrome: a community-based study of prevalence and impact. Br J Rheumatol 37(10):1069–1076

    Article  CAS  PubMed  Google Scholar 

  8. Martens PB, Pillemer SR, Jacobsson LT et al (1999) Survivorship in a population based cohort of patients with Sjögren’s syndrome, 1976–1992. J Rheumatol 26(6):1296–1300

    CAS  PubMed  Google Scholar 

  9. Brito-Zeron P, Ramos-Casals M, Bove A et al (2007) Predicting adverse outcomes in primary Sjögren’s syndrome: identification of prognostic factors. Rheumatology (Oxford) 46(8):1359–1362

    Google Scholar 

  10. Ioannidis JP, Vassiliou VA, Moutsopoulos HM (2002) Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren’s syndrome. Arthritis Rheum 46(3):741–747

    Article  PubMed  Google Scholar 

  11. Pertovaara M, Pukkala E, Laippala P et al (2001) A longitudinal cohort study of Finnish patients with primary Sjögren’s syndrome: clinical, immunological and epidemiological aspects. Ann Rheum Dis 60(5):467–472

    Article  CAS  PubMed  Google Scholar 

  12. Theander E, Manthorpe R, Jacobsson LT (2004) Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum 50(4):1262–1269

    Article  PubMed  Google Scholar 

  13. Seror R, Ravaud P, Bowman S et al (2009) EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI): Development of a consensus systemic disease activity index in primary Sjögren’s syndrome. Ann Rheum Dis

  14. Daniels TE, Criswell LA, Shiboski C et al (2009) An early view of the international Sjögren’s syndrome registry. Arthritis Rheum 61(5):711–714

    Article  PubMed  Google Scholar 

  15. Vitali C, Bombardieri S, Moutsopoulos HM et al (1993) Preliminary criteria for the classification of Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum 36(3):340–347

    Article  CAS  PubMed  Google Scholar 

  16. Vitali C, Bombardieri S, Moutsopoulos HM et al (1996) Assessment of the European classification criteria for Sjögren’s syndrome in a series of clinically defined cases: results of a prospective multicentre study. The European Study Group on Diagnostic Criteria for Sjögren’s Syndrome. Ann Rheum Dis 55(2):116–121

    Article  CAS  PubMed  Google Scholar 

  17. Zhang NZ, Shi CS, Yao QP et al (1995) Prevalence of primary Sjögren’s syndrome in China. J Rheumatol 22(4):659–661

    CAS  PubMed  Google Scholar 

  18. Manthorpe R, Oxholm P, Prause JU, Schiodt M (1986) The Copenhagen criteria for Sjögren’s syndrome. Scand J Rheumatol Suppl 61:19–21

    CAS  PubMed  Google Scholar 

  19. Fox RI, Robinson C, Curd J et al (1986) First international symposium on Sjögren’s syndrome: suggested criteria for classification. Scand J Rheumatol Suppl 61:28–30

    CAS  PubMed  Google Scholar 

  20. Haugen AJ, Peen E, Hulten B et al (2008) 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 37(1):30–34

    Article  CAS  PubMed  Google Scholar 

  21. Schein OD, Munoz B, Tielsch JM et al (1997) Prevalence of dry eye among the elderly. Am J Ophthalmol 124(6):723–728

    CAS  PubMed  Google Scholar 

  22. Schein OD, Hochberg MC, Munoz B et al (1999) Dry eye and dry mouth in the elderly: a population-based assessment. Arch Intern Med 159(12):1359–1363

    Article  CAS  PubMed  Google Scholar 

  23. Trontzas PI, Andrianakos AA (2005) Sjögren’s syndrome: a population based study of prevalence in Greece. The ESORDIG study. Ann Rheum 64(8):1240–1241

    Article  CAS  Google Scholar 

  24. Alamanos Y, Tsifetaki N, Voulgari PV et al (2006) Epidemiology of primary Sjögren’s syndrome in north-west Greece, 1982–2003. Rheumatology (Oxford) 45(2):187–191

    Google Scholar 

  25. Bowman SJ, Ibrahim GH, Holmes G et al (2004) Estimating the prevalence among Caucasian women of primary Sjögren’s syndrome in two general practices in Birmingham, UK. Scand J Rheumatol 33(1):39–43

    Article  CAS  PubMed  Google Scholar 

  26. Dafni UG, Tzioufas AG, Staikos P (1997) Prevalence of Sjögren’s syndrome in a closed rural community. Ann Rheum 56(9):521–525

    Article  CAS  Google Scholar 

  27. Tomsic M, Logar D, Grmek M et al (1999) Prevalence of Sjögren’s syndrome in Slovenia. Rheumatology (Oxford) 38(2):164–170

    Google Scholar 

  28. Kabasakal Y, Kitapcioglu G, Turk T et al (2006) The prevalence of Sjögren’s syndrome in adult women. Scand J Rheumatol 35(5):379–383

    Article  CAS  PubMed  Google Scholar 

  29. Pillemer SR, Matteson EL, Jacobsson LT et al (2001) Incidence of physician-diagnosed primary Sjögren syndrome in residents of Olmsted County, Minnesota. Mayo Clin Proc 76(6):593–599

    Article  CAS  PubMed  Google Scholar 

  30. Plesivcnik NM, Rozman B, Hocevar A et al (2004) Incidence of primary Sjögren’s syndrome in Slovenia. Ann Rheum Dis 63(7):874–876

    Article  Google Scholar 

  31. Pillemer SR, Matteson EL, Jacobsson LT et al (2001) Incidence of physician-diagnosed primary Sjögren syndrome in residents of Olmsted County, Minnesota. Mayo Clin Proc 76(6):593–599

    Article  CAS  PubMed  Google Scholar 

  32. Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM (2000) Clinical evolution and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum 29(5):296–304

    Article  CAS  PubMed  Google Scholar 

  33. Garcia-Carrasco M, Ramos-Casals M, Rosas J et al (2002) Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore) 81(4):270–280

    Google Scholar 

  34. Drosos AA, Tsiakou EK, Tsifetaki N et al (1997) Subgroups of primary Sjögren’s syndrome. Sjögren’s syndrome in male and paediatric Greek patients. Ann Rheum Dis 56(5):333–335

    Article  CAS  PubMed  Google Scholar 

  35. Brennan MT, Sankar V, Leakan RA et al (2002) Risk factors for positive minor salivary gland biopsy findings in Sjögren’s syndrome and dry mouth patients. Arthritis Rheum 47(2):189–195

    Article  PubMed  Google Scholar 

  36. Brennan MT, Fox PC (1999) Sex differences in primary Sjögren’s syndrome. J Rheumatol 26(11):2373–2376

    CAS  PubMed  Google Scholar 

  37. Gondran G, Fauchais A, Lambert M et al (2008) Primary Sjögren’s syndrome in men. Scand J Rheumatol 37(4):300–305

    Article  CAS  PubMed  Google Scholar 

  38. Horvath IF, Szodoray P, Zeher M (2008) Primary Sjögren’s syndrome in men: clinical and immunological characteristic based on a large cohort of Hungarian patients. Clin Rheumatol 27(12):1479–1483

    Article  PubMed  Google Scholar 

  39. Westhoff G, Edelmann E, Zink A (2009) Das Zuweiser-Projekt. Symptomdauer bis zur Erstvorstellung beim internistischen Rheumatologen. Rheuma-Management 1(2):13–15

    Google Scholar 

  40. Ramos-Casals M, Cervera R, Font J et al (1998) Young onset of primary Sjögren’s syndrome: clinical and immunological characteristics. Lupus 7(3):202–206

    Article  CAS  PubMed  Google Scholar 

  41. Westhoff G, Schneider M, Raspe H et al (2009) Advance and unmet need of health care for patients with rheumatoid arthritis in the German population-results from the German Rheumatoid Arthritis Population Survey (GRAPS). Rheumatology (Oxford) 48(6):650–657

    Google Scholar 

  42. Kruize AA, Hene RJ, Heide A van der et al (1996) Long-term followup of patients with Sjögren’s syndrome. Arthritis Rheum 39(2):297–303

    Article  CAS  PubMed  Google Scholar 

  43. Gannot G, Lancaster HE, Fox PC (2000) Clinical course of primary Sjögren’s syndrome: salivary, oral and serologic aspects. J Rheumatol 27(8):1905–1909

    CAS  PubMed  Google Scholar 

  44. Haldorsen K, Moen K, Jacobsen H et al (2008) Exocrine function in primary Sjögren syndrome: natural course and prognostic factors. Ann Rheum Dis 67(7):949–954

    Article  CAS  PubMed  Google Scholar 

  45. Pijpe J, Kalk WW, Bootsma H et al (2007) Progression of salivary gland dysfunction in patients with Sjögren’s syndrome. Ann Rheum Dis 66(1):107–112

    Article  CAS  PubMed  Google Scholar 

  46. Theander E, Henriksson G, Ljungberg O et al (2006) Lymphoma and other malignancies in primary Sjögren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis 65(6):796–803

    Article  CAS  PubMed  Google Scholar 

  47. Davidson BK, Kelly CA, Griffiths ID (1999) Primary Sjögren’s syndrome in the North East of England: a long-term follow-up study. Rheumatology (Oxford) 38(3):245–253

    Google Scholar 

  48. Masaki Y, Sugai S (2004) Lymphoproliferative disorders in Sjögren’s syndrome. Autoimmun Rev 3(3):175–182

    Article  PubMed  Google Scholar 

  49. Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM (1999) Malignant lymphoma in primary Sjögren’s syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren’s Syndrome. Arthritis Rheum 42(8):1765–1772

    Article  CAS  PubMed  Google Scholar 

  50. Zintzaras E, Voulgarelis M, Moutsopoulos HM (2005) The risk of lymphoma development in autoimmune diseases: a meta-analysis. Arch Intern Med 165(20):2337–2344

    Article  PubMed  Google Scholar 

  51. Theander E, Manthorpe R, Jacobsson LT (2004) Mortality and causes of death in primary Sjögren’s syndrome: a prospective cohort study. Arthritis Rheum 50(4):1262–1269

    Article  PubMed  Google Scholar 

  52. Ramos-Casals M, Brito-Zeron P, Yague J et al (2005) Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjögren’s syndrome. Rheumatology (Oxford) 44(1):89–94

    Google Scholar 

  53. Voulgarelis M, Tzioufas AG, Moutsopoulos HM (2008) Mortality in Sjögren’s syndrome. Clin Exp Rheumatol 26 (5 Suppl 51):S66–S71

    CAS  PubMed  Google Scholar 

  54. Skopouli FN, Dafni U, Ioannidis JP et al (2000) Clinical evolution and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum 29(5):296–304

    Article  CAS  PubMed  Google Scholar 

  55. Baboonian C, Venables PJ, Booth J (1989) Virus infection induces redistribution and membrane localization of the nuclear antigen La (SS-B): a possible mechanism for autoimmunity. Clin Exp Immunol 78(3):454–459

    CAS  PubMed  Google Scholar 

  56. Ramos-Casals M, Brito-Zeron P, Perez-De-Lis M et al (2009) Sjögren Syndrome or Sjögren Disease? The histological and immunological bias caused by the 2002 criteria. Clin Rev Allergy Immunol [Epub 6 July 2009]

  57. Galvez J, Saiz E, Lopez P et al (2009) Diagnostic evaluation and classification criteria in Sjögren’s Syndrome. Joint Bone Spine 76(1):44–49

    Article  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Forschungsbereich Epidemiologie, Deutsches Rheuma-Forschungszentrum Berlin (DRFZ), Charitéplatz 1, 10117, Berlin, Deutschland

    G. Westhoff Dipl.-Psych. & A. Zink

Authors
  1. G. Westhoff Dipl.-Psych.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. Zink
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to G. Westhoff Dipl.-Psych..

Rights and permissions

Reprints and permissions

About this article

Cite this article

Westhoff, G., Zink, A. Epidemiologie des primären Sjögren-Syndroms. Z. Rheumatol. 69, 41–49 (2010). https://doi.org/10.1007/s00393-009-0518-3

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00393-009-0518-3

Schlüsselwörter

Keywords

Search

Navigation