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Sjögren-Syndrom

Aktuelle Aspekte aus rheumatologischer Sicht

Sjögren’s syndrome

Current aspects from a rheumatological point of view

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Zusammenfassung

Das Sjögren-Syndrom ist eine autoimmune Erkrankung der exokrinen Drüsen mit den Leitsymptomen Keratokonjunktivitis und Stomatitis sicca auf Grundlage einer komplexen Pathogenese. Die Prävalenz beträgt ca. 0,5–1%, wobei ein primäres Sjögren-Syndrom von einem sekundären Sjögren-Syndrom bei anderen Autoimmunerkrankungen differenziert wird. Die Diagnose wird durch die subjektiven Beschwerden und den objektiven Nachweis von Sicca-Symptomen, Anti-Ro(SS-A)/La(SS-B)-Antikörpern und/oder einer fokalen lymphozytären Infiltration der Drüsengewebe gestellt. Neben der typischen Sicca-Symptomatik, die symptomatisch durch Substitutions- und Stimulationstherapie behandelt wird, weisen einige Patienten extraglanduläre Manifestationen auf. Dominierend finden sich Beschwerden am Bewegungsapparat und an inneren Organen, die durch den Rheumatologen behandelt werden. Die Indikation zur Basistherapie wird individuell gestellt; deren Wirksamkeit ist jedoch nicht durch Studien belegt. Etwa 5–10% der Patienten mit einem primären Sjögren-Syndrom entwickeln ein Non-Hodgkin-Lymphom der B-Zell-Reihe. Die Erkrankung erfordert eine interdisziplinäre Betreuung mit Augen-, Zahn-, HNO- und anderen Kollegen in Abhängigkeit vom klinischen Bild.

Abstract

Sjögren’s syndrome is an autoimmune disease of the exocrine glands characterized by the leading symptoms of keratoconjunctivitis and stomatitis sicca based on a complex pathogenesis. The prevalence is about 0.5–1%; primary Sjögren’s syndrome is differentiated from secondary Sjögren’s syndrome associated with other autoimmune disorders. The diagnosis is established by the presence of subjective complaints and objective evidence of sicca symptoms, anti-Ro(SSA)/La(SSB) antibodies, and/or focal lymphocytic infiltration of the glandular tissue. In addition to the typical sicca symptomatology, which is managed symptomatically by substitution and stimulation therapy, some patients exhibit extraglandular manifestations. Complaints involving the musculoskeletal system and inner ear dominate and are treated by the rheumatologist. The indication for base therapy is tailored to individual needs, but the efficacy of this approach has not been established in studies. About 5–10% of the patients with primary Sjögren’s syndrome develop a B-cell non-Hodgkin’s lymphoma. The disease requires interdisciplinary management including, among others, ophthalmologists, dentists, and otorhinolaryngologists, depending on the clinical picture.

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Literatur

  1. Ackerman LS (2006) Sex hormones and the genesis of autoimmunity. Arch Dermatol 142(3): 371–376

    Article  PubMed  Google Scholar 

  2. Al-Hashimi I (2005) Xerostomia secondary to Sjögren’s syndrome in the elderly. Drugs Aging 22(11): 887–899

    Article  PubMed  Google Scholar 

  3. Aragona P, Di Pietro R, Spinella R, Mobrici M (2006) Conjunctival epithelium improvement after systemic pilocarpine in patients with Sjogren’s syndrome. Br J Ophthalmol 90(2): 166–170

    Article  PubMed  Google Scholar 

  4. Asmussen K, Oxholm P (1999) Disease activity in primary Sjögren’s syndrome – can it be defined and assessed? State of the art lectures on Sjögren’s syndrome. Hygiea 108 (part 1) http://www.sjogrensyndrom.se/100-site/index100.html

  5. Asmussen KH, Bowman SJ (2001) Outcome measures in Sjögren’s syndrome. Rheumatology 40: 1085–1088

    Article  PubMed  Google Scholar 

  6. Bernatsky S, Ramsey-Goldman R, Clarke A (2006) Malignancy and autoimmunity. Curr Opin Rheumatol 18(2): 129–134

    Article  PubMed  Google Scholar 

  7. Bloch KJ, Buchanan WW, Wohl MJ, Bunim JJ (1965) Sjögren’s syndrome, a clinical, pathological and serological study of sixty-two cases. Medicine 44: 187–231

    PubMed  Google Scholar 

  8. Bolstad AI, Jonsson R (2002) Genetic aspects of Sjögren’s syndrome. Arthritis Res 4: 353–359

    Article  PubMed  Google Scholar 

  9. Bowman SJ, Booth DA, Platts RG (2004) Measurement of fatigue and discomfort in primary Sjögren’s syndrome using a new questionnaire tool. Rheumatology 43: 758–764

    Article  PubMed  Google Scholar 

  10. Bowman SJ, Pillemer S, Jonsson R et al. (2001) Revisiting Sjögren’s syndrome in the new millennium: perspectives on assessment and outcome measures. Report of a workshop held on 23 March 2000 at Oxford, UK. Rheumatology 40: 1180–1188

    Article  PubMed  Google Scholar 

  11. Bredberg A, Henriksson G, Larsson Å et al. (2004) Sjögren’s syndrome and the Danger model. Rheumatol 44: 965–970

    Article  Google Scholar 

  12. Bredberg A, Manthorpe R, Henriksson G (2005) Recent findings shed light on the aethiopathogenesis of Sjögren’s syndrome. Akt Rheumatol 30: 1–4

    Article  Google Scholar 

  13. Brennan MT, Sankar V, Leakan RA et al. (2003) Sex steroid hormones in primary Sjogren’s syndrome. J Rheumatol 30(6): 1267–1271

    PubMed  Google Scholar 

  14. Bron AJ, Tiffany JM, Gouveia SM et al. (2004) Functional aspects of the tear film lipid layer. Exp Eye Res 78(3): 347–360

    Article  PubMed  Google Scholar 

  15. Callaghan R, Prabu A, Allan RB et al. (2006) Direct healthcare costs and predictors of costs in patients with primary Sjögren’s syndrome. Rheumatology 25 (Epub ahead of print)

  16. Cha S, Singson E, Cornelius J et al. (2006) Muscarinic acetylcholine type-3 receptor desensitization due to chronic exposure to Sjogren’s syndrome-associated autoantibodies. J Rheumatol 33(2): 296–306

    PubMed  Google Scholar 

  17. Champey J, Corruble E, Gottenberg JE et al. (2006) Quality of life and psychological status in patients with primary Sjogren’s syndrome and sicca symptoms without autoimmune features. Arthritis Rheum 55(3): 451–457

    Article  PubMed  Google Scholar 

  18. Chisholm DM, Mason DK (1968) Labial salivary gland biopsy in Sjögren’s disease. J Clin Pathol 21: 656–660

    PubMed  Google Scholar 

  19. Cimaz R, Casadei A, Rose C et al. (2003) Primary Sjogren syndrome in the paediatric age: a multicentre survey. Eur J Pediatr 162(10): 661–665

    Article  PubMed  Google Scholar 

  20. Cummins MJ, Papas A, Kammer GM, Fox PC (2003) Treatment of primary Sjögren’s syndrome with low-dose human interferon-α administered by the oromucosal route: combined phase III results. Arthritis Rheum 49:585–593

    Article  PubMed  Google Scholar 

  21. Cutolo M, Sulli A, Capellino S et al. (2004) Sex hormones influence on the immune system: basic and clinical aspects in autoimmunity. Lupus 13(9): 635–638

    Article  PubMed  Google Scholar 

  22. Dalavanga YA, Detrick B, Hooks JJ et al. (1987) Effect of cyclosporin A (CyA) on the immunopathological lesion of the labial minor salivary glands from patients with Sjogren’s syndrome. Ann Rheum Dis 46(2): 89–92

    PubMed  Google Scholar 

  23. Dartt DA (2002) Regulation of mucin and fluid secretion by conjunctival epithelial cells. Progress Retinal Eye Res 21: 555–576

    Article  Google Scholar 

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

    Google Scholar 

  25. Dawson LJ, Stanbury J, Venn N et al. (2006) Antimuscarinic antibodies in primary Sjogren’s syndrome reversibly inhibit the mechanism of fluid secretion by human submandibular salivary acinar cells. Arthritis Rheum 54(4): 1165–1173

    Article  PubMed  Google Scholar 

  26. Diener HC, Putzki N, Berlit P et al. (2005) Diagnostik und Therapie der vaskulitischen Neuropathien und Neuropathien bei Kollagenosen; Leitlinien für Diagnostik und Therapie in der Neurologie, 3. überarbeitete Aufl. Georg Thieme, Stuttgart, http://www.uni-duesseldorf.de/awmf/ll/030–068.htm

  27. Dörner T (2001) Neonatale Lupussyndrome. In: Zeidler H, Zacher J, Hiepe F (Hrsg) Interdisziplinäre klinische Rheumatologie. Springer, Berlin Heidelberg New York Tokyo, S 865–894

  28. Dörner T (2001) Sjögren Syndrom. In: Zeidler H, Zacher J, Hiepe F (Hrsg) Interdisziplinäre klinische Rheumatologie. Springer, Berlin Heidelberg New York Tokyo, S 922–928

  29. Dörner T, Gromnica-Ihle E (2006) Sjögren-Syndrom, Kapitel 4.1. In: Rationelle Diagnostik und Therapie in der Inneren Medizin. Elsevier/Urban Fischer, München Jena

  30. Dörner T, Lipsky PE (2002) Abnormalities of B cell phenotype, immunoglobulin gene expression and the emergence of autoimmunity in Sjögren’s syndrome. Arthritis Res 4: 360–371

    Article  PubMed  Google Scholar 

  31. Drosos AA, Andonopoulos AP, Costopoulos JS et al. (1988) Prevalence of primary Sjögren’s syndrome in an elderly population. Rheumatology 27: 123–127

    Google Scholar 

  32. Drosos AA, Skopouli FN, Costopoulos JS et al. (1986) Cyclosporin A (CyA) in primary Sjogren’s syndrome: a double blind study. Ann Rheum Dis 45(9): 732–735

    PubMed  Google Scholar 

  33. Drosos AA, Skopouli FN, Galanopoulou VK et al. (1986) Cyclosporin a therapy in patients with primary Sjogren’s syndrome: results at one year. Scand J Rheumatol Suppl 61: 246–249

    PubMed  Google Scholar 

  34. Fife RS, Chase WF, Dore RK et al. (2002) Cevimeline for the treatment of xerostomia in patients with Sjögren syndrome: a randomized trial. Arch Internal Med 162: 1293–1300

    Article  Google Scholar 

  35. Fox PC, Datiles M, Atkinson JC et al. (1993) Prednisone and Piroxicam for treatment of primary Sjögren’s syndrome. Clin Exp Rheumatol 11: 149–156

    PubMed  Google Scholar 

  36. Fox RI, Chan E, Benton L (1986) Treatment of primary Sjögren’s syndrome with hydroxychloroquine. Am J Med 85 [Suppl 4A]: 62–70

  37. Fox RI, Dixon R, Guarrasi V, Krubel S (1996) Treatment of primary Sjögren’s syndrome with hydroxychloroquin: a retrospective, open-label study. Lupus 5 [Suppl 1]: S31–S36

  38. Godaert GLR, Hartkamp A, Geenen R et al. (2002) Fatigue in daily life in patients with primary Sjögren’s syndrome and systemic lupus erythematosus. Ann NY Acad Sci 966: 320–326

    PubMed  Google Scholar 

  39. Waterman SA, Gordon TP, Rischmueller M (2000) Inhibitory effects of muscarinic receptor autoantibodies on parasympathetic neurotransmission in Sjogren’s syndrome. Arthritis Rheum 43(7): 1647–1654

    Article  PubMed  Google Scholar 

  40. Gottenberg JE, Guillevin L, Lambotte O et al. (2005) Tolerance and short term efficacy of rituximab in 43 patients with systemic autoimmune diseases. Ann Rheum Dis 64(6): 913–920

    Article  PubMed  Google Scholar 

  41. Groom J, Kalled SL, Cutler AH et al. (2002) Association of BAFF/BLyS overexpression and altered B cell differentiation with Sjogren’s syndrome. J Clin Invest 109(1): 59–68

    Article  PubMed  Google Scholar 

  42. Gudbjörnsson B, Broman JE, Hetta J, Hällgren R (1993) Sleep disturbances in patients with primary Sjögren’s syndrome. Rheumatology 32: 1072–1076

    Google Scholar 

  43. Hamilton JB (1943) A new conception of Keratoconjunctivitis sicca (keratitis filiformis in hypofunction of the lacrimal glands). Australasian Medical Publishing Company, Sydney

  44. Hansen A, Gosemann M, Pruss A et al. (2004) Abnormalities in peripheral B cell memory of patients with primary Sjögren’s syndrome. Arthritis Rheum 50: 1897–1908

    Article  PubMed  Google Scholar 

  45. Hansen A, Hoffmann A, Dörner T (2005) Sjögren-Syndrom – können wir das Lymphomrisiko stratifizieren? Akt Rheumatol 30: 46–49

    Article  Google Scholar 

  46. Hansen A, Lipsky PE, Dorner T (2005) Immunopathogenesis of primary Sjogren’s syndrome: implications for disease management and therapy. Curr Opin Rheumatol 17(5): 558–565

    Article  PubMed  Google Scholar 

  47. Hansen A, Odendahl M, Reiter K et al. (2002) Disturbed B cell homeostasis in Sjögren’s syndrome: indication for the accumulation of memory B cells in the salivary glands. Arthritis Rheum 46: 2160–2171

    Article  PubMed  Google Scholar 

  48. Hansen A, Reiter K, Pruss A et al. (2006) Dissemination of a Sjögren‘s syndrome-associated extranodal marginal zone B cell lymphoma. Arthritis Rheum 54: 127–137

    Article  PubMed  Google Scholar 

  49. Höld KM, de Boer D, Zuidema J, Maes RAA (1995) Saliva as an analytical tool in toxicology. Int J Drug Testing 1(1): 1–36 (http://www.criminology.fsu.edu/journal/hold.pdf)

    Google Scholar 

  50. 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 

  51. Isenberg DA (2006) B cell targeted therapies in autoimmune diseases. J Rheumatol Suppl 77: 24–28

    PubMed  Google Scholar 

  52. Ittah M, Miceli-Richard C, Eric Gottenberg J et al. (2006) B cell-activating factor of the tumor necrosis factor family (BAFF) is expressed under stimulation by interferon in salivary gland epithelial cells in primary Sjögren’s syndrome. Arthritis Res Therapy 8: R51

    Article  Google Scholar 

  53. Kassan SS, Thomas TL, Moutsopoulos HM et al. (1978) Increased risk of lymphoma in sicca syndrome. Ann Intern Med 89(6): 888–892

    PubMed  Google Scholar 

  54. Kassi E, Moutsatsou P, Sekeris CE et al. (2003) Oestrogen receptors in cultured epithelial cells from salivary glands of Sjögren’s syndrome patients. Rheumatology 42: 1120–1122

    Article  PubMed  Google Scholar 

  55. Kauppi M, Pukkala L, Isomäki H (1997) Elevated incidence of hematologic malignancies in patients with Sjögren’s syndrome compared with patients, with rheumatoid arthritis (Finland). Cancer Causes Control 8: 201–214

    Article  PubMed  Google Scholar 

  56. Kruize AA, Héne RJ, Kallenberg CGM et al. (1993) Hydroxychloroquine treatment for primary Sjögren’s syndrome: a two-year, double-blind cross-over trial. Ann Rheum Dis 52: 360–364

    PubMed  Google Scholar 

  57. Lazarus MN, Robinson D, Mak V et al. (2006) Incidence of cancer in a cohort of patients with primary Sjögren’s syndrome. Rheumatology, Advance Access published on February 20, 2006. doi: 10.1093/rheumatology/kei281

  58. Leng XM, Zhao Y, Zhou DB et al. (2005) A pilot trial for severe, refractory systemic autoimmune disease with stem cell transplantation. Chin Med Sci J 20(3): 159–165 (abstract)

    PubMed  Google Scholar 

  59. Lipsky PE, Diamond B (2006) Autoimmunity and autoimmune diseases, Chapter 8. In: Fauci AS (ed) Harrison’s rheumatology. McGraw-Hill Medical Publishing Division, New York, p 63

  60. Manthorpe R (2005) Wie klassifizieren wir Patienten, bei den wir ein Sjögren-Syndrom vermuten? Geschichtliche Entwicklung verschiedener Klassifikationen und vermutliche Weiterentwicklung. Akt Rheumatol 30: 32–37

    Article  Google Scholar 

  61. Manthorpe R, Benoni C, Jacobsson L et al. (2000) Lower frequency of focal lip sialadenitis (focus score) in smoking patients. Can tobacco diminish the salivary gland involvement as judged by histological examination and anti-SSA/Ro and anti-SSB/La antibodies in Sjogren’s syndrome? Ann Rheum Dis 59(1): 54–60

    Article  PubMed  Google Scholar 

  62. Manthorpe R, Manthorpe T (2005) Das primäre Sjögren-Syndrom – Epidemiologie und Prognose. Akt Rheumatol 30: 27–31

    Article  Google Scholar 

  63. Mariette X, Ravaud P, Steinfeld S et al. (2004) Inefficacy of infliximab in primary Sjogren’s syndrome: results of the randomized, controlled Trial of Remicade in Primary Sjogren’s Syndrome (TRIPSS). Arthritis Rheum 50(4): 1270–1276

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  65. Messmer EM (2005) Differenzierte Therapie der Keratoconjunctivitis sicca beim Sjögren-Syndrom. Akt Rheumatol 30: 59–65

    Article  Google Scholar 

  66. Mitsias DI, Tzioufas G, Veiopoulou C et al. (2002) The Th1/Th2 cytokine balance changes with the progress of the immunological lesion of Sjogren’s syndrome. Clin Exp Immunol 128: 562–568

    Article  PubMed  Google Scholar 

  67. Moutsopoulos HM (2006) Sjögren’s syndrom, Chapter 8. In: Fauci AS (ed) Harrison’s rheumatology. McGraw-Hill Medical Publishing Division, New York, pp 133–138

  68. Niederkorn JY, Stern ME, Pflugfelder SC et al. (2006) Desiccating stress induces T cell-mediated Sjogren’s Syndrome-like lacrimal keratoconjunctivitis. J Immunol 176(7): 3950–3957

    PubMed  Google Scholar 

  69. Noble BA, Loh RSK, MacLennan S et al. (2004) Comparison of autologous serum eye drops with conventional therapy in a randomised controlled crossover trial for ocular surface disease. Br J Opthalmol 88: 647–652

    Article  Google Scholar 

  70. Ono M, Takamura E, Shinozaki K et al. (2004) Therapeutic effect of cevimeline on dry eye in patients with Sjogren’s syndrome: a randomized, double-blind clinical study. Am J Ophthalmol 138(1): 6–17

    Article  PubMed  Google Scholar 

  71. Palombi G, the Study Group for SjS of the Italian Society of Rheumatology (2006) Sjögren’s syndrome Disease Damage Index (SjSDDI) a scoring system for the quantification of damage in SjS, derived by the analysis of a cohort of Italian patients. Posterpräsentation IX. International Symposium on Sjogren’s Syndrom Washighton DC 27.–29.4.2006

  72. Paulsen FP, Schaudig U, Thale AB (2003) Drainage of tears: impact on the ocular surface and lacrimal system. Clin Sci 1(4): 180–191

    Google Scholar 

  73. Pertovaara M, Antonen J, Hurme M (2006) Th2 cytokine genotypes are associated with a milder form of primary Sjogren’s syndrome. Ann Rheum Dis 65(5): 666–670

    Article  PubMed  Google Scholar 

  74. 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: 467–472

    Article  PubMed  Google Scholar 

  75. Pijpe J, van Imhoff GW, Spijkervet FK et al. (2005) Rituximab treatment in patients with primary Sjogren’s syndrome: an open-label phase II study. Arthritis Rheum 52(9): 2740–2750

    Article  PubMed  Google Scholar 

  76. Pillemer SR, Brennan MT, Sankar V et al. (2004) Pilot clinical trial of dehydroepiandrosterone (DHEA) versus placebo for Sjogren’s syndrome. Arthritis Rheum 51(4): 601–604

    Article  PubMed  Google Scholar 

  77. Pleyer U, Dörner T (2001) Sjögren Syndrom – systemische und ophthalmologische Manifestationen. Uni-Med Taschenbuch Augenheilkunde, 1. Aufl. Uni-Med, Bremen, S 80–89, 103–104

  78. Prause JU, Frost-Larsen K, Hoj L et al. (1984) Lacrimal and salivary secretion in Sjogren’s syndrome: the effect of systemic treatment with bromhexine. Acta Ophthalmol (Copenh) 62(3): 489–497

    Google Scholar 

  79. Price EJ, Rigby SP, Clancy U, Venables PJ (1998) Double blind placebo controlled trial of azathioprine in the treatment of primary Sjogren’s syndrome. J Rheumatol 25(5): 896–899

    PubMed  Google Scholar 

  80. Ramos-Casals M, Font J (2005) Primary Sjögren’s syndrome: current and emergent aetiopathogenic concepts. Rheumatology 44: 1354–1367

    Article  PubMed  Google Scholar 

  81. Ramos-Casals M, Nardi N, Brito-Zeron P et al. (2006) Atypical autoantibodies in patients with primary Sjogren syndrome: clinical characteristics and follow-up of 82 cases. Semin Arthritis Rheum 35(5): 312–321

    Article  PubMed  Google Scholar 

  82. Reinikainen LM, Jaakkola JJ (2003) Significance of humidity and temperature on skin and upper airway symptoms. Indoor Air 13(4): 344–352

    Article  PubMed  Google Scholar 

  83. Rosenthal P, Cotter J (2003) The Boston Scleral Lens in the management of severe ocular surface disease. Ophthalmol Clin North Am 16(1): 89–93

    Article  PubMed  Google Scholar 

  84. Salomonsson S, Jonsson MV, Skarstein K et al. (2003) Cellular basis of ectopic germinal center formation and autoantibody production in the target organ of patients with Sjögren’s syndorm. Arthritis Rheum 48(11): 3187–3201

    Article  PubMed  Google Scholar 

  85. Sánchez-Guerrero J, Pérez-Dosal MR, Cárdenas-Velázquez F et al. (2005) Prevalence of Sjögren’s syndrome in ambulatory patients according to the American-European Consensus Group criteria. Rheumatology 44: 235–240

    PubMed  Google Scholar 

  86. Sankar V, Brennan MT, Kok MR et al. (2004) Etanercept in Sjögren’s syndrome: a twelve-week randomized, double-blind, placebo-controlled pilot clinical trial. Arthritis Rheum 50: 2240–2245

    Article  PubMed  Google Scholar 

  87. Scott D, Hiepe F, Hummel M et al. (1998) Antigen-driven clonal proliferation of B cells within the target tissue of an autoimmune disease. The salivary glands of patients with Sjogren’s syndrome. J Clin Invest 102(5): 938–946

    PubMed  Google Scholar 

  88. Sharpe M, Wilks D (2002) ABC of psychological medicine: Fatigue. BMJ 325: 480–483

    Article  PubMed  Google Scholar 

  89. Sjögren HSC (1933) Zur Kenntnis der Keratoconjunctivitis sicca (Keratitis filiformis bei Hypofunktion der Tränendrüsen). Acta Ophthalmologica, Copenhagen [Suppl II]: 1–151

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

    Article  PubMed  Google Scholar 

  91. Skopouli FN, Jagiello P, Tsifetaki N, Moutsopoulos HM (1996) Methotrexate in primary Sjogren’s syndrome. Clin Exp Rheumatol 14(5): 555–558

    PubMed  Google Scholar 

  92. Stangel M, Gold Rolf für die IVIg-Konsensusgruppe: Einsatz intravenöser Immunglobuline in der Neurologie – ein evidenzbasierter Konsens, S. 29. http://www.dgn.org/fileadmin/leitl/ivigkonsensgruppe.pdf

  93. Steinfeld SD, Demols P, Appelboom T (2002) Infliximab in primary Sjogren’s syndrome: one-year followup. Arthritis Rheum 46(12): 3301–3303

    Article  PubMed  Google Scholar 

  94. Steinfeld SD, Demols P, Salmon I et al. (2001) Infliximab in patients with primary Sjogren’s syndrome: a pilot study. Arthritis Rheum 44(10): 2371–2375

    Article  PubMed  Google Scholar 

  95. Steinfeld SD, Tant L, Burmester GR et al. (2006) Epratzumab (humanized anti-CD22 antibody) in primary Sjogren’s syndrome: an open-label Phase I/II study. Arthritis Res Ther 8: R129 (http://arthritis-research.com/content/pdf/ar2018.pdf)

    Article  PubMed  Google Scholar 

  96. Stern ME, Beuerman RW, Fox RI et al. (1998) The pathology of dry eye: the interaction between the ocular surface and lacrimal glands. Cornea 17(6): 584–589

    Article  PubMed  Google Scholar 

  97. Stiller M (2005) Die orale Komponente des Sjögren-Syndroms – diagnostische Aspekte und zahnmedizinisch-therapeutische Konsequenzen. Akt Rheumatol 30: 41–45

    Article  Google Scholar 

  98. Stroehmann A, Dörner T, Lukowsky A et al. (2002) Cutaneous T cell lymphoma in a patient with primary biliary cirrhosis and secondary Sjögren’s syndrome. J Rheumatol 29: 1326–1330

    PubMed  Google Scholar 

  99. Strömbeck B (2006) Women with primary Sjögren’s syndrome; assessment and treatment; a physiotherapeutic perspective. Doctoral Thesis, Lund University

  100. Sullivan DA, Sullivan BD, Ullman MD et al. (2000) Androgen influence on the Meibomian gland. Invest Ophthalmol Vis Sci 41: 3732–3742

    PubMed  Google Scholar 

  101. Sutcliffe NN, Inanc M, Speight P, Isenberg D (1998) Predictors of lymphoma development in primary Sjögren’s syndrome. Semin Arthritis Rheum 28: 80–87

    Article  PubMed  Google Scholar 

  102. Szodoray P, Jonsson R (2005) The BAFF/APRIL system in systemic autoimmune diseases with a special emphasis on Sjögren’s syndrome. Scand J Immunol 62(5): 421–428

    Article  PubMed  Google Scholar 

  103. Tabbara KF, Frayha RA (1993) Alternate-day steroid therapy for patients with primary Sjögren’s syndrome. Ann Ophthal 15: 358–361

    Google Scholar 

  104. Talal N, Bunim JJ (1964) The development of malignant lymphoma in the course of Sjögren’s syndrome. Am J Med 36: 529–540

    Article  PubMed  Google Scholar 

  105. Theander E (2005) Living and dying with primary Sjögren’s syndrome. Studies on aetiology, treatment, lymphoma, survival and predictors. Thesis. Lund University, Malmö, pp 1–144

  106. Theander E, Andersson SI, Manthorpe R, Jocobsson LTH (2005) Proposed core set of outcome measures in patients with primary Sjögren’s syndrome. A 5 years follow-up. J Rheumatol 32: 1495–1502

    PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  109. Tishler M, Yaron I, Shirazi I, Yaron M (1999) Hydroxychloroquine treatment for primary Sjogren’s syndrome: its effect on salivary and serum inflammatory markers. Ann Rheum Dis 58: 253–256

    PubMed  Google Scholar 

  110. Tsifetaki N, Kitsos G, Paschides CA et al. (2003) Oral pilocarpine for the treatment of ocular symptoms in patients with Sjogren’s syndrome: a randomised 12 week controlled study. Ann Rheum Dis 62(12): 1204–1207

    Article  PubMed  Google Scholar 

  111. Valesini G, Priori R, Bavoillot D et al. (1997) Differential risk of non-Hodgkin’s lymphoma in Italian patients with primary Sjogren’s syndrome. J Rheumatol 24(12): 2376–2380

    PubMed  Google Scholar 

  112. Valimaa H, Savolainen S, Soukka T et al. (2004) Estrogen receptor-beta is the predominant subtype in human oral epithelium and salivary glands. J Endocrinol 180(1): 55–62

    Article  PubMed  Google Scholar 

  113. Valtiýsdóttir ST, Gudjbjörnsson B, Hällgren R, Hetta J (2000) Psychological well-being in patients with primary Sjögren’s syndrome. Clin Exp Rheumatol 18: 597–600

    PubMed  Google Scholar 

  114. van Woerkom J, Kruize AA, Jacobs KMG et al. (2006) Clinical efficacy of leflunomide in the treatment of primary Sjögren’s syndrome correlates with a decrease in pro-inflammatory cytokine production. SAT0105 EULAR 2006

  115. van Woerkom J, Kruize AA, Jacobs KMG et al. (2005) Safety and efficacy of leflunomide in primary Sjögren’s syndrome: preliminary results of a pilot study. FRI0172 EULAR 2005

  116. 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 America-European-Consensus Group. Ann Rheum Dis 61: 554–558

    Article  PubMed  Google Scholar 

  117. Vitali C, Palombi G, the Study Group for SjS of the Italian Society of Rheumatology (2006) Sjögren’s syndrome Disease Activity Measurement (SjSDAM): a scoring system fort he assessment of disease acitivty in SjS, derived by the analysis of a cohort of Italian patients. Posterpräsentation IX. International Symposium on Sjogren’s Syndrom Washighton DC 27.–29.4.2006

  118. Vivino FB, Al-Hashimi I, Khan Z et al. (1999) Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjögren syndrome: a randomized, placebo-controlled, fixed-dose, multicenter trial. Arch Internal Med 159: 174–181

    Article  Google Scholar 

  119. Voulgarelis M, Giannouli S, Tzioufas AG, Moutsopoulos HM (2005) Long-term remission of Sjogren’s syndrome-associated aggressive B-cell non-Hodgkin’s lymphomas following administration of combined B-cell depletion therapy and CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone). Ann Rheum Dis published Online First: 1 December 2005. doi: 10.1136/ard.2005.046193

  120. Youinou P, Moutsopoulos HM, Pennec YL (1990) Clinical features of Sjogren’s syndrome. Curr Opin Rheumatol 2(5): 687–693

    PubMed  Google Scholar 

  121. Zandbelt MM, Blokx WA, DeJong EM et al. (2006) Expression of estrogen receptors alpha and beta in skin and salivary gland biospy specimens of Sjögren’s syndrome patients. Posterpräsentation EULAR-Kongress Amsterdam SAT 0110

  122. Zandbelt MM, de Wilde P, van Damme P et al. (2004) Etanercept in the treatment of patients with primary Sjogren’s syndrome: a pilot study. J Rheumatol 31(1): 96–101

    PubMed  Google Scholar 

  123. 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 

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Tomiak, C., Dörner, T. Sjögren-Syndrom. Z. Rheumatol. 65, 505–519 (2006). https://doi.org/10.1007/s00393-006-0101-0

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