Best Practice & Research Clinical Rheumatology
Volume 21, Issue 6 , Pages 989-1010 , December 2007

Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias

  • Athanasios G. Tzioufas, MD (Associate Professor)

      Affiliations

    • Corresponding Author InformationCorresponding author. Department of Pathophysiology, School of Medicine, University of Athens, 75 M. Asias Street, 11527 Athens, Greece. Tel.: +30 210 7462670; Fax: +30 210 7462664.
  • ,
  • Michael Voulgarelis, MD (Assistant Professor)

References 

  1. Dafni U, Tzioufas AG, Staikos P, et al. The prevalence of Sjögren's syndrome in a close rural community. Annals of the Rheumatic Diseases. 1997;56:521–525
  2. Jacobsson L, Axell TE, Hansen B, et al. Dry eyes or mouth – an epidemiological study in Swedish adults with special reference to primary Sjögren's syndrome. Journal of Autoimmunity. 1989;2:521–527
  3. Moutsopoulos HM. Sjögren's syndrome autoimmune epithelitis. Clinical Immunology and Immunopathology. 1994;72:162–165
  4. Mitsias DI, Kapsogeorgou EK, Moutsopoulos HM. Sjögren's syndrome: why autoimmune epithelitis?. Oral Diseases. 2006;12:523–532
  5. Daniels TE, Whitcher JP. Association of patterns of labial salivary gland inflammation with keratoconjunctivitis sicca. Analysis of 618 patients with suspected Sjögren's syndrome. Arthritis & Rheumatism. 1994;37:869–877
  6. Salomonsson S, Jonsson MV, Skarstein K, et al. Cellular basis of ectopic germinal center formation and autoantibody production in the target organ of patients with Sjögren's syndrome. Arthritis & Rheumatism. 2003;48:3187–3201
  7. Pavlidis NA, Karsh J, Moutsopoulos HM. The clinical picture of primary Sjögren's syndrome: a retrospective study. The Journal of Rheumatology. 1982;9:685–690
  8. Al-Hashimi I, Khuder S, Haghighat N, Zipp M. Frequency and predictive value of the clinical manifestations in Sjögren's syndrome. Journal of Oral Pathology & Medicine. 2001;30:1–6
  9. Boutsi EA, Paikos S, Dafni UG, et al. Dental and periodontal status of Sjögren's syndrome. Journal of Clinical Periodontology. 2000;27:231–235
  10. Daniels TE, Fox PC. Salivary and oral components of Sjögren's syndrome. Rheumatic Diseases Clinics of North America. 1992;18:571–589
  11. Niemela RK, Takalo R, Paakko E, et al. Ultrasonography of salivary glands in primary Sjögren's syndrome. A comparison with magnetic resonance imaging and magnetic resonance sialography of parotid glands. Rheumatology (Oxford). 2004;43:875–879
  12. Morimoto Y, Habu M, Tomoyose T, et al. Dynamic magnetic resonance sialography as a new diagnostic technique for patients with Sjögren's syndrome. Oral Diseases. 2006;12:408–414
  13. Samarkos M, Moutsopoulos HM. Recent advances in the management of ocular complications of Sjögren's syndrome. Current Allergy and Asthma Reports. 2005;5:327–332
  14. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Archives of Internal Medicine. 2004 Jun 28;164(12):1275–1284
  15. Castro-Poltronieri A, Alarcon-Segovia D. Articular manifestations of primary Sjögren's syndrome. The Journal of Rheumatology. 1983;10:485–488
  16. Skopouli FN, Talal A, Galanopoulou V, et al. Raynaud's phenomenon in primary Sjögren's syndrome. The Journal of Rheumatology. 1990;17:618–620
  17. Fox RI, Liu AY. Sjögren's syndrome in dermatology. Clinics in Dermatology. 2006;24:393–413
  18. Papiris SA, Maniati M, Constantopoulos SH, et al. Lung involvement in primary Sjögren's syndrome is mainly related to the small airway disease. Annals of the Rheumatic Diseases. 1999;58:61–64
  19. Ito I, Nagai S, Kitaichi M, et al. Kitaichi M,et al Pulmonary manifestations of primary Sjögren's syndrome: a clinical, radiologic, and pathologic study. American Journal of Respiratory and Critical Care Medicine. 2005;15(171):632–638
  20. Trevino H, Tsianos EB, Schenker S. Gastrointestinal and hepatobiliary features in Sjögren's syndrome. In:  Talal N,  Moutsopoulos HM,  Kassan SS editor. Sjögren's syndrome: clinical and immunological aspects. Berlin: Springer-Verlag; 1987;p. 89–95
  21. Skopouli FN, Barbatis C, Moutsopoulos HM. Liver involvement in primary Sjögren's syndrome. British Journal of Rheumatology. 1994;33:745–748
  22. Goules A, Masouridi S, Tzioufas AG, et al. Clinically significant and biopsy-documented renal involvement in primary Sjögren syndrome. Medicine (Baltimore). 2000;79:241–249
  23. Pertovaara M, Korpela M, Kouri T, Pasternack A. The occurrence of renal involvement in primary Sjögren's syndrome: a study of 78 patients. Rheumatology (Oxford). 1999;38:1113–1120
  24. Moutsopoulos HM, Balow JE, Lawley TJ, et al. Immune complex glomerulonephritis in sicca syndrome. American Journal of Medicine. 1978;64:955–960
  25. Tsokos M, Lazarou SA, Moutsopoulos HM. Vasculitis in primary Sjögren's syndrome: histologic classification and clinical presentation. American Journal of Clinical Pathology. 1987;88:26–31
  26. Alexander EL, Arnett FC, Provost TT, Stevens MB. Sjögren's syndrome: association of anti-Ro(SS-A) antibodies with vasculitis, hematologic abnormalities, and serologic hyperreactivity. Annals of Internal Medicine. 1983;98:155–159
  27. Gemignani F, Marbini A, Pavesi G, et al. Peripheral neuropathy associated with primary Sjögren's syndrome. Journal of Neurology, Neurosurgery, and Psychiatry. 1994;57:983–986
  28. Kanellopoulos P, Baltoyiannis C, Tzioufas AG. Primary Sjögren's syndrome associated with inclusion body myositis. Rheumatology (Oxford). 2002;41:440–444
  29. Alexander GE, Provost TT, Stevens MB, Alexander EL. Sjögren's syndrome: central nervous system manifestations. Neurology. 1981;31:1391–1396
  30. Ioannidis JP, Moutsopoulos HM. Sjögren's syndrome: too many associations, too limited evidence. The enigmatic example of CNS involvement. Seminars in Arthritis and Rheumatism. 1999;29:1–3
  31. Karsh J, Pavlidis N, Weintraub BD, Moutsopoulos HM. Thyroid disease in Sjögren's syndrome. Arthritis & Rheumatism. 1980;23:1326–1329
  32. Ramos-Casals M, Garcia-Carrasco M, Cervera R, et al. Thyroid disease in primary Sjögren syndrome. Study in a series of 160 patients. Medicine (Baltimore). 2000;79:103–108
  33. Tapinos NI, Polihronis M, Tzioufas AG, Moutsopoulos HM. Sjögren's syndrome. Autoimune epithelitis. Advances In Experimental Medicine and Biology. 1999;455:127–134
  34. Haneji N, Nakamura T, Takio K, et al. Identification of alpha-fodrin as a candidate autoantigen in primary Sjögren's syndrome. Science. 1997;276:604–607
  35. Kuwana M, Okano T, Ogawa Y, et al. Autoantibodies to the amino-terminal fragment of beta-fodrin expressed in glandular epithelial cells in patients with Sjögren's syndrome. Journal of Immunology. 2001;167:5449–5456
  36. Bacman S, Perez Leiros C, Sterin-Borda L, et al. Autoantibodies against lacrimal gland M3 muscarinic acetylcholine receptors in patients with primary Sjögren's syndrome. Investigative Ophthalmology & Visual Science. 1998;39:151–156
  37. Feist E, Kuckelkorn U, Dorner T, et al. Autoantibodies in primary Sjögren's syndrome are directed against proteasomal subunits of the alpha and beta type. Arthritis & Rheumatism. 1999;42:697–702
  38. Mavragani CP, Tzioufas AG, Moutsopoulos HM. Sjögren's syndrome: autoantibodies to cellular antigens. Clinical and molecular aspects. International Archives of Allergy and Applied Immunology. 2000;123:46–57
  39. Manoussakis MN, Tzioufas AG, Pange PJ, Moutsopoulos HM. Serological profiles in subgroups of patients with Sjögren's syndrome. Scandinavian Journal of Rheumatology Supplement. 1986;61:89–92
  40. Moutsopoulos HM, Webber BL, Vlagopoulos TP, et al. Differences in the clinical manifestations of sicca syndrome in the presence and absence of rheumatoid arthritis. American Journal of Medicine. 1979;66:733–736
  41. Manoussakis MN, Georgopoulou C, Zintzaras E, et al. Sjögren's syndrome associated with systemic lupus erythematosus: clinical and laboratory profiles and comparison with primary Sjögren's syndrome. Arthritis & Rheumatism. 2004;50:882–891
  42. Andonopoulos AP, Drosos AA, Skopouli FN, Moutsopoulos HM. Sjögren's syndrome in rheumatoid arthritis and progressive systemic sclerosis. A comparative study. Clinical and Experimental Rheumatology. 1989;7:203–205
  43. Mavragani CP, Moutsopoulos NM, Moutsopoulos HM. The management of Sjögren's syndrome. Nature Clinical Practice. Rheumatology. 2006;2:252–261
  44. Fries JF, Hochberg MC, Medsger TA, et al. Criteria for rheumatic diseases. Different types and functions. Arthritis & Rheumatism. 1994;37:454–462
  45. Vitali C, Bombardieri S. Diagnostic criteria for Sjögren's syndrome: the state of the art. Clinical and Experimental Rheumatology. 1990;8(supplement 5):13–16
  46. Vitali C, Bombardieri S, Moutsopoulos HM, et al. Preliminary criteria for the classification of Sjögren's syndrome. Results of a prospective concerted action supported by the European Community. Arthritis & Rheumatism. 1992;36:340–348
  47. Vitali C, Moutsopoulos HM, Bombardieri S. The European Community Study Group on diagnostic criteria for Sjögren's syndrome. Sensitivity and specificity of tests for ocular and oral involvement in Sjögren's syndrome. Annals of the Rheumatic Diseases. 1994;53:637–647
  48. Vitali C, Bombardieri S, Moutsopoulos HM, et al. 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. Annals of the Rheumatic Diseases. 1996;55:116–121
  49. 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. Annals of the Rheumatic Diseases. 2002;61:554–558
  50. Rubin H, Holt M. Secretory sialography in diseases of the major salivary glands. AJR American Journal of Roentgenology. 1957;77:575–598
  51. Shall GL, Anderson LG, Wolf RO, et al. Xerostomia in Sjögren's syndrome: evaluation by sequential scintigraphy. Journal of the American Medical Association. 1971;216:2109–2116
  52. Drosos AA, Constantopoulos SH, Psychos D, et al. The forgotten cause of sicca complex; sarcoidosis. The Journal of Rheumatology. 1989;16:1548–1551
  53. Itescu S, Braneato LJ, Buxbaum J, et al. A diffuse infiltrative CD8 lymphocytosis syndrome in human immunodeficiency virus (HIV) infection: a host immune response associated with HLA-DR5. Annals of Internal Medicine. 1990;112:3–10
  54. Haddad J, Deny P, Munz-Gothiel C, et al. Lymphocytic sialadenitis of Sjögren's syndrome associated with chronic hepatitis C virus liver disease. Lancet. 1992;339:321–323
  55. Vitali C, Sciuto M, Neri R, et al. Anti hepatitis C virus antibodies in primary Sjögren's syndrome: false positive results are related to hyper-g-globulinemia. Clinical and Experimental Rheumatology. 1992;10:103–104
  56. Ramos-Casals M, Loustaud-Ratti V, De Vita S, et al. Sjögren syndrome associated with hepatitis C virus: a multicenter analysis of 137 cases. Medicine (Baltimore). 2005;84:81–89
  57. Mackay IR, Rose NR. Autoimmunity and lymphoma: tribulations of B cells. Nature Immunology. 2001;2:793–795
  58. Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases: a meta-analysis. Archives of Internal Medicine. 2005;165:2337–2344
  59. Kassan SS, Thomas TL, Moutsopoulos HM, et al. Increased risk of lymphoma in sicca syndrome. Annals of Internal Medicine. 1978;89:888–892
  60. Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren's syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome. Arthritis & Rheumatism. 1999;42:1765–1772
  61. Royer B, Cazals-Hatem D, Sibilia J, et al. Lymphomas in patients with Sjögren's syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nolal sites and are not associated with viruses. Blood. 1997;90:766–775
  62. Mariette X. Lymphomas in patients with Sjögren's syndrome: review of the literature and physiopathologic hypothesis. Leukemia & Lymphoma. 1999;33(1–2):93–99
  63. Theander E, Henriksson G, Ljungberg O, et al. Lymphoma and other malignancies in primary Sjögren's syndrome: a cohort study on cancer incidence and lymphoma predictors. Annals of the Rheumatic Diseases. 2006;65:796–803
  64. Smedby KE, Hjalgrim H, Askling J, et al. Autoimmune and chronic inflammatory disorders and risk of non-Hodgkin lymphoma by subtype. Journal of the National Cancer Institute. 2006;98:51–60
  65. Pelstring RJ, Essell JH, Kurtin PJ, et al. Diversity of organ site involvement among malignant lymphomas of mucosa-associated tissues. American Journal of Clinical Pathology. 1991;96:738–745
  66. Dogan A, Du M, Koulis A, et al. Expression of lymphocyte homing receptors and vascular addressins in low-grade gastric B-cell lymphomas of mucosa-associated lymphoid tissue. American Journal of Pathology. 1997;151:1361–1369
  67. Harris NL, Isaacson PG. What are the criteria for distinguishing MALT from non-MALT lymphoma at extranodal sites?. American Journal of Clinical Pathology. 1999;111(1 Suppl 1):S126–S132
  68. Jaffe ES. Lymphoid lesions of the head and neck: a model of lymphocyte homing and lymphomagenesis. Modern Pathology. 2002;15:255–263
  69. Rossi D, Gaidano G. Molecular heterogeneity of diffuse large B-cell lymphoma: implications for disease management and prognosis. Hematology. 2002;7:239–252
  70. Anderson LG, Talal N. The spectrum of benign to malignant lymphoproliferation in Sjögren's syndrome. Clinical and Experimental Immunology. 1972;10:199–221
  71. 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 & Rheumatism. 1996;39:767–772
  72. Walters MT, Stevenson FK, Herbert A, et al. Urinary monoclonal free light chains in the primary Sjögren's syndrome: an aid to the diagnosis of malignant lymphoma. Annals of the Rheumatic Diseases. 1986;45:210–219
  73. Skopouli FN, Dafni U, Ioannidis JP, Moutsopoulos HM. Clinical evolution, and morbidity and mortality of primary Sjögren's syndrome. Seminars in Arthritis and Rheumatism. 2000;29:296–304
  74. Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren's syndrome. Arthritis & Rheumatism. 2002;46:741–747
  75. 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
  76. Sutcliffe N, Inanc M, Speight P, Isenberg D. Predictors of lymphoma development of primary Sjögren's syndrome. Seminars in Arthritis and Rheumatism. 1998;28:80–87
  77. Anaya JM, McGuff HS, Banks PM, Talal N. Clinicopathological factors relating malignant lymphoma with Sjögren's syndrome. Seminars in Arthritis and Rheumatism. 1996;25:337–346
  78. Cheema GS, Roschke V, Hilbert DM, Stohl W. Elevated serum B lymphocyte stimulator levels in patients with systemic immune-based rheumatic diseases. Arthritis & Rheumatism. 2001;44:1313–1319
  79. Groom J, Kalled SL, Cutler AH, et al. Association of BAFF/BLyS overexpression and altered B cell differentiation with Sjögren's syndrome. The Journal of Clinical Investigation. 2002;109:59–68
  80. Jacobi AM, Odendahl M, Reiter K, et al. Correlation between circulating CD27high plasma cells and disease activity in patients with systemic lupus erythematosus. Arthritis & Rheumatism. 2003;48:1332–1342
  81. Bohnhorst JO, Thoen JE, Natvig JB, Thompson KM. Significantly depressed percentage of CD27+ (memory) B cells among peripheral blood B cells in patients with primary Sjögren's syndrome. Scandinavian Journal of Immunology. 2001;54:421–427
  82. Hansen A, Odendahl M, Reiter K, et al. Diminished peripheral blood memory B cells and accumulation of memory B cells in the salivary glands of patients with Sjögren's syndrome. Arthritis & Rheumatism. 2002;46:2160–2171
  83. Zulman J, Jaffe R, Talal N. Evidence that the malignant lymphoma of Sjögren's syndrome is a monoclonal B-cell neoplasm. The New England Journal of Medicine. 1978;299:1215–1220
  84. Theander E, Manthorpe R, Jacobsson LT. Mortality and causes of death in primary Sjögren's syndrome: a prospective cohort study. Arthritis & Rheumatism. 2004;50:1262–1269
  85. Zinzani PL, Magagnoli M, Galieni P, et al. Nongastrointestinal low-grade mucosa-associated lymphoid tissue lymphoma: analysis of 75 patients. Journal of Clinical Oncology. 1999;17:1254–1258
  86. Zucca E, Conconi A, Pedrinis E, et al. Nongastric marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue. Blood. 2003;101:2489–2495
  87. Thieblemont C, Berger F, Dumontet C, et al. Mucosa-associated lymphoid tissue lymphoma is a disseminated disease in one third of 158 patients analyzed. Blood. 2000;95(3):802–806
  88. Voulgarelis M, Moutsopoulos HM. Malignant lymphoma in primary Sjögren's syndrome. The Israel Medical Association Journal. 2001;3:761–766
  89. Voulgarelis M, Petroutsos G, Moutsopoulos HM, Skopouli FN. 2-chloro-2-deoxyadenosine in the treatment of Sjögren's syndrome-associated B cell lymphoproliferation. Arthritis & Rheumatism. 2002;46:2248–2249
  90. Pijpe J, van Imhoff GW, Spijkervet FK, et al. Rituximab treatment in patients with primary Sjögren's syndrome: an open-label phase II study. Arthritis & Rheumatism. 2005;52:2740–2750
  91. Pijpe J, van Imhoff GW, Vissink A, et al. Changes in salivary gland immunohistology and function after rituximab monotherapy in a patient with Sjögren's syndrome and associated MALT lymphoma. Annals of the Rheumatic Diseases. 2005;64:958–960
  92. Voulgarelis M, Giannouli S, Anagnostou D, Tzioufas AG. Combined therapy with rituximab plus cyclophosphamide/doxorubicin/vincristine/prednisone (CHOP) for Sjögren's syndrome-associated B-cell aggressive non-Hodgkin's lymphomas. Rheumatology (Oxford). 2004;43:1050–1053
  93. Voulgarelis M, Giannouli S, Tzioufas AG, Moutsopoulos HM. Long-term remission of Sjögren's syndrome-associated aggressive B-cell non-Hodgkin's lymphomas following administration of combined B-cell depletion therapy and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). Annals of the Rheumatic Diseases. 2006;65:1033–1037

PII: S1521-6942(07)00105-2

doi: 10.1016/j.berh.2007.09.001

Best Practice & Research Clinical Rheumatology
Volume 21, Issue 6 , Pages 989-1010 , December 2007