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Cancer

  • Elke Theander
  • Eva Baecklund
Chapter

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].

Keywords

Systemic Lupus Erythematosus Salivary Gland Hodgkin Lymphoma Malt Lymphoma Standardize Incidence Ratio 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

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.

References

  1. 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.PubMedCrossRefGoogle Scholar
  2. 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.PubMedCrossRefGoogle Scholar
  3. 3.
    Theander E, Manthorpe R, Jacobsson LTH. Mortality and causes of death in primary Sjögren’s syndrome. Arthritis Rheum. 2004;50:1262–9.PubMedCrossRefGoogle Scholar
  4. 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.PubMedCrossRefGoogle Scholar
  5. 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.PubMedCrossRefGoogle Scholar
  6. 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.PubMedCrossRefGoogle Scholar
  7. 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.PubMedCrossRefGoogle Scholar
  8. 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.PubMedGoogle Scholar
  9. 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.PubMedCrossRefGoogle Scholar
  10. 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.PubMedCrossRefGoogle Scholar
  11. 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. 12.
    Kassan SS, Thomas TL, Moutsopoulos HM, et al. Increased risk of lymphoma in sicca syndrome. Ann Intern Med. 1978;89:888–92.PubMedGoogle Scholar
  13. 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.PubMedCrossRefGoogle Scholar
  14. 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.PubMedCrossRefGoogle Scholar
  15. 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.PubMedCrossRefGoogle Scholar
  16. 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.PubMedCrossRefGoogle Scholar
  17. 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.CrossRefGoogle Scholar
  18. 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.PubMedCrossRefGoogle Scholar
  19. 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.PubMedCrossRefGoogle Scholar
  20. 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.PubMedCrossRefGoogle Scholar
  21. 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.CrossRefGoogle Scholar
  22. 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.PubMedCrossRefGoogle Scholar
  23. 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.PubMedCrossRefGoogle Scholar
  24. 24.
    Anderson LG, Talal N. The spectrum of benign to malignant lymphoproliferation in Sjögren’s syndrome. Clin Exp Immunol. 1972;10:199–221.PubMedGoogle Scholar
  25. 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.PubMedCrossRefGoogle Scholar
  26. 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.PubMedGoogle Scholar
  27. 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.PubMedCrossRefGoogle Scholar
  28. 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.PubMedCrossRefGoogle Scholar
  29. 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.CrossRefGoogle Scholar
  30. 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.PubMedCrossRefGoogle Scholar
  31. 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.PubMedCrossRefGoogle Scholar
  32. 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.PubMedGoogle Scholar
  33. 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.PubMedGoogle Scholar
  34. 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.PubMedCrossRefGoogle Scholar
  35. 35.
    Cook M, Bareford D, Kumararatne D. Non-Hodgkin’s lymphoma: an unusual complication of idiopathic CD4+-lymphocytopenia. Hosp Med. 1998;59:582.PubMedGoogle Scholar
  36. 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.PubMedGoogle Scholar
  37. 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.PubMedCrossRefGoogle Scholar
  38. 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.PubMedGoogle Scholar
  39. 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.PubMedGoogle Scholar
  40. 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.PubMedCrossRefGoogle Scholar
  41. 41.
    Hansen A, Lipsky PE, Dorner T. B-cell lymphoproliferation in chronic inflammatory rheumatic diseases. Nat Clin Pract Rheumatol. 2007;3:561–9.PubMedCrossRefGoogle Scholar
  42. 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.PubMedCrossRefGoogle Scholar
  43. 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.PubMedCrossRefGoogle Scholar
  44. 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.PubMedCrossRefGoogle Scholar
  45. 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.PubMedCrossRefGoogle Scholar
  46. 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.PubMedCrossRefGoogle Scholar
  47. 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.PubMedCrossRefGoogle Scholar
  48. 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.PubMedCrossRefGoogle Scholar
  49. 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.PubMedCrossRefGoogle Scholar
  50. 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.PubMedCrossRefGoogle Scholar
  51. 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.CrossRefGoogle Scholar
  52. 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.PubMedGoogle Scholar
  53. 53.
    Frisch M, Melbye M. New primary cancers after squamous cell skin cancer. Am J Epidemiol. 1995;141:916–22.PubMedGoogle Scholar
  54. 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.PubMedGoogle Scholar
  55. 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.PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag London Limited 2011

Authors and Affiliations

  • Elke Theander
    • 1
  • Eva Baecklund
    • 2
  1. 1.Department of RheumatologySkåne University HospitalMalmöSweden
  2. 2.Rheumatology Unit, Department of Medical SciencesUppsala UniversityUppsalaSweden

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