Skip to main content

Cancer

  • Chapter
  • First Online:
Sjögren’s Syndrome
  • 1191 Accesses

Abstract

Sjögren’s syndrome (SS) is a chronic, systemic, inflammatory rheumatic disease that demonstrates features of organ-specific and non-organ-specific autoimmunity [1]. SS can occur in a primary form (pSS) or in secondary form (sSS) in association with various other rheumatic diseases, primarily rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) [2]. In SLE, the term “associated disorder” or overlap syndrome may be more appropriate than “secondary” SS because of the similarities of pathogenic processes and clinical manifestations in the two diseases and the difficulties in differentiating reliably between them in a considerable ­number of patients [2].

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

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 84.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 139.00
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 109.99
Price excludes VAT (USA)
  • Durable hardcover 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. Jonsson R, Bolstad AI, Brokstad KA, Brun JG. Sjogren’s syndrome – a plethora of clinical and immunological phenotypes with a complex genetic background. Ann N Y Acad Sci. 2007;1108:433–47.

    Article  PubMed  CAS  Google Scholar 

  2. Theander E, Jacobsson LT. Relationship of Sjogren’s syndrome to other connective tissue and autoimmune disorders. Rheum Dis Clin North Am. 2008;34:935–47.

    Article  PubMed  Google Scholar 

  3. Theander E, Manthorpe R, Jacobsson LTH. Mortality and causes of death in primary Sjögren’s syndrome. Arthritis Rheum. 2004;50:1262–9.

    Article  PubMed  Google Scholar 

  4. Vitali C, Bombardieri S, Jonsson R, et al. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis. 2002;61:554–8.

    Article  PubMed  CAS  Google Scholar 

  5. Smedby KE, Baecklund E, Askling J. Malignant lymphomas in autoimmunity and inflammation: a review of risks, risk factors, and lymphoma characteristics. Cancer Epidemiol Biomarkers Prev. 2006;15:2069–77.

    Article  PubMed  CAS  Google Scholar 

  6. Baecklund E, Iliadou A, Askling J, et al. Association of chronic inflammation, not its treatment, with increased lymphoma risk in rheumatoid arthritis. Arthritis Rheum. 2006;54:692–701.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  8. Goransson LG, Haldorsen K, Brun JG, et al. The point prevalence of clinically relevant primary Sjogren’s syndrome in two Norwegian counties. Scand J Rheumatol 2011;40:221–4.

    PubMed  Google Scholar 

  9. Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjogren’s syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjogren’s Syndrome. Arthritis Rheum. 1999;42:1765–72.

    Article  PubMed  CAS  Google Scholar 

  10. Theander E, Henriksson G, Ljungberg O, Mandl T, Manthorpe R, Jacobsson LT. Lymphoma and other malignancies in primary Sjögren’s syndrome: a cohort study on cancer incidence and lymphoma predictors. Ann Rheum Dis. 2006;65:796–803.

    Article  PubMed  CAS  Google Scholar 

  11. Swerdlow SH, Campo E, Harris NL, et al., editors. Pathology and genetics: tumours of the haematopoietic and lymphoid tissue. Lyon: IARC Press; 2008.

    Google Scholar 

  12. Kassan SS, Thomas TL, Moutsopoulos HM, et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med. 1978;89:888–92.

    PubMed  CAS  Google Scholar 

  13. Smedby KE, Hjalgrim H, Askling J, et al. Autoimmune and chronic inflammatory disorders and risk of non-Hodgkin lymphoma by subtype. J Natl Cancer Inst. 2006;98:51–60.

    Article  PubMed  Google Scholar 

  14. 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;46:741–7.

    Article  PubMed  Google Scholar 

  15. Anderson LA, Gadalla S, Morton LM, et al. Population-based study of autoimmune conditions and the risk of specific lymphoid malignancies. Int J Cancer. 2009;125:398–405.

    Article  PubMed  CAS  Google Scholar 

  16. Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases: a meta-analysis. Arch Intern Med. 2005;165:2337–44.

    Article  PubMed  Google Scholar 

  17. Zhang W, Feng S, Yan S, et al. Incidence of malignancy in primary Sjogren’s syndrome in a Chinese cohort. Rheumatology (Oxford). 2010;49:571–7.

    Article  Google Scholar 

  18. Ramos-Casals M, Cervera R, Font J, et al. Young onset of primary Sjogren’s syndrome: clinical and immunological characteristics. Lupus. 1998;7:202–6.

    Article  PubMed  CAS  Google Scholar 

  19. Sutcliffe N, Inanc M, Speight P, Isenberg D. Predictors of lymphoma development in primary Sjögren’s syndrome. Semin Arthritis Rheum. 1998;28:80–7.

    Article  PubMed  CAS  Google Scholar 

  20. Ekstrom Smedby K, Vajdic CM, Falster M, et al. Autoimmune disorders and risk of non-Hodgkin lymphoma subtypes: a pooled analysis within the InterLymph Consortium. Blood. 2008;111:4029–38.

    Article  PubMed  Google Scholar 

  21. Baimpa E, Dahabreh IJ, Voulgarelis M, Moutsopoulos HM. Hematologic manifestations and predictors of lymphoma development in primary Sjogren syndrome: clinical and pathophysiologic aspects. Medicine (Baltimore). 2009;88:284–93.

    Article  Google Scholar 

  22. Voulgarelis M, Moutsopoulos HM. Mucosa-associated lymphoid tissue lymphoma in Sjögren’s syndrome: risks, management, and prognosis. Rheum Dis Clin North Am. 2008;34:921–33.

    Article  PubMed  Google Scholar 

  23. Friedman J, Schattner A, Shvidel L, Berrebi A. Characterization of T-cell large granular lymphocyte leukemia associated with Sjogren’s syndrome-an important but under-recognized association. Semin Arthritis Rheum. 2006;35:306–11.

    Article  PubMed  CAS  Google Scholar 

  24. Anderson LG, Talal N. The spectrum of benign to malignant lymphoproliferation in Sjögren’s syndrome. Clin Exp Immunol. 1972;10:199–221.

    PubMed  CAS  Google Scholar 

  25. Anaya JM, McGuff S, Banks PM, Talal N. Clinicopathological factors relating malignant lymphoma with Sjögren’s syndrome. Semin Arthritis Rheum. 1996;25:337–46.

    Article  PubMed  CAS  Google Scholar 

  26. Barone F, Bombardieri M, Rosado MM, et al. CXCL13, CCL21, and CXCL12 expression in salivary glands of patients with Sjogren’s syndrome and MALT lymphoma: association with reactive and malignant areas of lymphoid organization. J Immunol. 2008;180:5130–40.

    PubMed  CAS  Google Scholar 

  27. Skopouli FN, Dafni U, Ioannidis JPA, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum. 2000;29:296–304.

    Article  PubMed  CAS  Google Scholar 

  28. Tzioufas AG, Boumba DS, Skopouli FN, Moutsopoulos HM. Mixed monoclonal cryoglobulinemia and monoclonal rheumatoid factor cross-reactive idiotypes as predictive factors for the development of lymphoma in primary Sjögren’s syndrome. Arthritis Rheum. 1996;39:767–72.

    Article  PubMed  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  30. Walters MT, Stevenson FK, Herbert A, Cawley MI, Smith JL. Urinary monoclonal free light chains in primary Sjogren’s syndrome: an aid to the diagnosis of malignant lymphoma. Ann Rheum Dis. 1986;45:210–9.

    Article  PubMed  CAS  Google Scholar 

  31. 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;60:467–72.

    Article  PubMed  CAS  Google Scholar 

  32. Kirtava Z, Blomberg J, Bredberg A, Henriksson G, Jacobsson L, Manthorpe R. CD4+ T-lymphocytopenia without HIV infection: increased prevalence among patients with primary Sjögren’s syndrome. Clin Exp Rheumatol. 1995;13:609–16.

    PubMed  CAS  Google Scholar 

  33. Mandl T, Bredberg A, Jacobsson LT, Manthorpe R, Henriksson G. CD4+ T-lymphocytopenia – a frequent finding in anti-SSA antibody seropositive patients with primary Sjogren’s syndrome. J Rheumatol. 2004;31:726–8.

    PubMed  Google Scholar 

  34. Hanamura I, Wakita A, Harada S, et al. Idiopathic CD4+ T-lymphocytopenia in a non-­Hodgkin’s lymphoma patient. Intern Med. 1997;36:643–6.

    Article  PubMed  CAS  Google Scholar 

  35. Cook M, Bareford D, Kumararatne D. Non-Hodgkin’s lymphoma: an unusual complication of idiopathic CD4+-lymphocytopenia. Hosp Med. 1998;59:582.

    PubMed  CAS  Google Scholar 

  36. Guilloton L, Drouet A, Bernard P, Berbineau A, Berger F, Kopp N. Cerebral intravascular lymphoma during T-CD4+ idiopathic lymphopenia syndrome. Presse Med. 1999;28:1513–5.

    PubMed  CAS  Google Scholar 

  37. Christodoulou MI, Kapsogeorgou EK, Moutsopoulos NM, Moutsopoulos HM. Foxp3+ T-regulatory cells in Sjogren’s syndrome: correlation with the grade of the autoimmune lesion and certain adverse prognostic factors. Am J Pathol. 2008;173:1389–96.

    Article  PubMed  Google Scholar 

  38. Theander E, Vasaitis L, Baecklund E, et al. Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjogren’s syndrome. Ann Rheum Dis 2011;70:1363–68.

    PubMed  Google Scholar 

  39. Jonsson MV, Skarstein K, Jonsson R, Brun JG. Serological implications of germinal center-like structures in primary Sjogren’s syndrome. J Rheumatol. 2007;34:2044–9.

    PubMed  Google Scholar 

  40. Küppers R, Klein U, Hansmann M-L, Rajewsky K. Cellular origin of human B-cell lymphomas. N Engl J Med. 1999;341:1520–9.

    Article  PubMed  Google Scholar 

  41. Hansen A, Lipsky PE, Dorner T. B-cell lymphoproliferation in chronic inflammatory rheumatic diseases. Nat Clin Pract Rheumatol. 2007;3:561–9.

    Article  PubMed  CAS  Google Scholar 

  42. Ghesquieres H, Berger F, Felman P, et al. Clinicopathologic characteristics and outcome of diffuse large B-cell lymphomas presenting with an associated low-grade component at diagnosis. J Clin Oncol. 2006;24:5234–41.

    Article  PubMed  Google Scholar 

  43. Novak AJ, Grote DM, Stenson M, et al. Expression of BLyS and its receptors in B-cell non-Hodgkin lymphoma: correlation with disease activity and patient outcome. Blood. 2004;104:2247–53.

    Article  PubMed  CAS  Google Scholar 

  44. De Vita S, Quartuccio L, Fabris M. Hepatitis C virus infection, mixed cryoglobulinemia and BLyS upregulation: targeting the infectious trigger, the autoimmune response, or both? Autoimmun Rev. 2008;8:95–9.

    Article  PubMed  Google Scholar 

  45. Mariette X. Lymphomas complicating Sjögren’s syndrome and hepatitis C virus infection may share a common pathogenesis: chronic stimulation of rheumatoid factor B cells. Ann Rheum Dis. 2001;60:1007–10.

    Article  PubMed  CAS  Google Scholar 

  46. Seror R, Ravaud P, Bowman S, et al. EULAR Sjogren’s syndrome disease activity index (ESSDAI): development of a consensus systemic disease activity index in primary Sjogren’s syndrome. Ann Rheum Dis. 2010;69:1103–9.

    Article  PubMed  Google Scholar 

  47. Meijer J, Meiners P, Vissink A, et al. Effective rituximab treatment in primary Sjogren’s syndrome: a randomised, double-blind, placebo-controlled trial. Arthritis Rheum. 2010;62:960–8.

    Article  PubMed  CAS  Google Scholar 

  48. Pijpe J, Meijer JM, Bootsma H, et al. Clinical and histologic evidence of salivary gland ­restoration supports the efficacy of rituximab treatment in Sjogren’s syndrome. Arthritis Rheum. 2009;60:3251–6.

    Article  PubMed  CAS  Google Scholar 

  49. Kauppi M, Pukkala E, Isomäki H. Elevated incidence of hematologic malignancies in patients with Sjögren’s syndrome compared with patients with rheumatoid arthritis (Finland). Cancer Causes Control. 1997;8:201–4.

    Article  PubMed  CAS  Google Scholar 

  50. Löfström B, Backlin C, Sundstrom C, Ekbom A, Lundberg I. A closer look at non-Hodgkin’s lymphoma cases in a national Swedish systemic lupus erythematosus cohort: a nested case-control study. Ann Rheum Dis. 2007;66:1627–32.

    Article  PubMed  Google Scholar 

  51. Lazarus MN, Robinson D, Mak V, Moller H, Isenberg DA. Incidence of cancer in a cohort of patients with primary Sjogren’s syndrome. Rheumatology (Oxford). 2006;45:1012–5.

    Article  CAS  Google Scholar 

  52. Bernatsky S, Boivin JF, Joseph L, et al. Prevalence of factors influencing cancer risk in women with lupus: social habits, reproductive issues, and obesity. J Rheumatol. 2002;29:2551–4.

    PubMed  Google Scholar 

  53. Frisch M, Melbye M. New primary cancers after squamous cell skin cancer. Am J Epidemiol. 1995;141:916–22.

    PubMed  CAS  Google Scholar 

  54. Frisch M, Hjalgrim H, Olsen JH, Melbye M. Risk for subsequent cancer after diagnosis of basal-cell carcinoma. A population-based, epidemiologic study. Ann Intern Med. 1996;125:815–21.

    PubMed  CAS  Google Scholar 

  55. Wassberg C, Thorn M, Yuen J, Ringborg U, Hakulinen T. Second primary cancers in patients with squamous cell carcinoma of the skin: a population-based study in Sweden. Int J Cancer. 1999;80:511–5.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

The authors are grateful to Prof. Otto Ljungberg and Dr. Malin V. Jonsson for providing photographs of B cell lymphomas and germinal center-like structures in the salivary glands and Prof. Küppers for allowing the reproduction of the cartoon on B cell development within the Germinal Centers.

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer-Verlag London Limited

About this chapter

Cite this chapter

Theander, E., Baecklund, E. (2011). Cancer. In: Ramos-Casals, M., Stone, J., Moutsopoulos, H. (eds) Sjögren’s Syndrome. Springer, London. https://doi.org/10.1007/978-0-85729-947-5_33

Download citation

  • DOI: https://doi.org/10.1007/978-0-85729-947-5_33

  • Published:

  • Publisher Name: Springer, London

  • Print ISBN: 978-0-85729-946-8

  • Online ISBN: 978-0-85729-947-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics