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Sjögren-Syndrom

Sjögren’s syndrome

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Zusammenfassung

Das primäre Sjögren-Syndrom ist mit einer Prävalenz zwischen 1:100 und 1:1000 die häufigste Kollagenose. Dennoch ist die Erkrankung wegen der überwiegend unspezifischen Symptome und der bei vielen Patienten fehlenden spezifischen Marker oft schwer zu diagnostizieren. Zudem wurden bislang nur wenige kontrollierte Therapiestudien durchgeführt, sodass die optimale Behandlung noch nicht klar ist. Mittlerweile wurden aber Outcomeparameter für den Therapieerfolg etabliert, sodass aktuell eine große Zahl klinischer Studien läuft. Im Review werden die aktuellen Diagnostik- und Behandlungsmöglichkeiten des primären Sjögren-Syndroms zusammengefasst.

Abstract

The prevalence of primary Sjögren’s syndrome (pSS) is between 1:100 and 1:1000 and it is therefore the most common connective tissue disease. Nevertheless, it can be difficult to diagnose pSS as the symptoms are frequently unspecific and diagnostic markers are lacking in many patients. In addition, only few controlled therapeutic studies of pSS have been carried out so that the optimal management is not yet clear. Meanwhile, outcome parameters to monitor clinical improvement have been developed and a large number of therapeutic studies are currently being performed. This review article summarizes the current diagnostic and treatment options for pSS.

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Literatur

  1. Adamson TC 3rd, Fox RI et al (1983) Immunohistologic analysis of lymphoid infiltrates in primary Sjogren’s syndrome using monoclonal antibodies. J Immunol 130:203–208

    PubMed  Google Scholar 

  2. 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 47:189–195

    Article  Google Scholar 

  3. Carsons SE, Vivino FB, Parke A et al (2017) Treatment guidelines for rheumatologic manifestations of Sjögren’s syndrome: use of biologic agents, management of fatigue, and inflammatory musculoskeletal pain. Arthritis Care Res (hoboken) 69:517–527

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  5. Cornec D, Jousse-Joulin S, Pers JO et al (2013) Contribution of salivary gland ultrasonography to the diagnosis of Sjögren’s syndrome: toward new diagnostic criteria? Arthritis Rheum 65:216–225

    Article  Google Scholar 

  6. Gottenberg JE, Ravaud P, Puéchal X et al (2014) Effects of hydroxychloroquine on symptomatic improvement in primary Sjögren syndrome: the JOQUER randomized clinical trial. JAMA 312:249–258

    Article  CAS  Google Scholar 

  7. Kohler PF, Winter ME (1985) A quantitative test for xerostomia. The Saxon test, an oral equivalent of the Schirmer test. Arthritis Rheum 28:1128–1132

    Article  CAS  Google Scholar 

  8. Meijer JM, Meiners PM, Vissink A et al (2010) Effectiveness of rituximab treatment in primary Sjögren’s syndrome: a randomized, double-blind, placebo-controlled trial. Arthritis Rheum 62:960–968

    Article  CAS  Google Scholar 

  9. Qin B, Wang J, Yang Z et al (2015) Epidemiology of primary Sjögren’s syndrome: a systematic review and meta-analysis. Ann Rheum Dis 74:1983–1989

    Article  CAS  Google Scholar 

  10. Radstake TRDJ, van der Heijden EHM, Moret FM et al (2018) Clinical efficacy of leflunomide/Hydroxychloroquine combination therapy in patients with primary Sjogren’s syndrome: results of a placebo-controlled double-blind randomized clinical trial. Arthritis Rheumatol 70(suppl 10). https://acrabstracts.org/abstract/clinical-efficacy-of-leflunomide-hydroxychloroquine-combination-therapy-in-patients-with-primary-sjogrens-syndrome-results-of-a-placebo-controlled-double-blind-randomized-clinical-trial

  11. Robinson CP, Brayer J, Yamachika S et al (1998) Transfer of human serum IgG to nonobese diabetic Igmu null mice reveals a role for autoantibodies in the loss of secretory function of exocrine tissues in Sjögren’s syndrome. Proc Natl Acad Sci U S A 95:7538–7543

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  14. Seror R, Ravaud P, Bowman SJ (2010) EULAR Sjogren’s syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren’s syndrome. Ann Rheum Dis 69:1103–1109

    Article  Google Scholar 

  15. Seror R, Ravaud P, Mariette X et al (2011) EULAR Sjogren’s Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sjogren’s syndrome. Ann Rheum Dis 70:968–972

    Article  Google Scholar 

  16. Shiboski CH, Shiboski SC, Seror R et al (2017) International Sjögren’s Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren’s syndrome: a consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 76:9–16

    Article  Google Scholar 

  17. Shiboski SC, Shiboski CH, Criswell L (2012) American College of Rheumatology classification criteria for Sjögren’s syndrome: a data-driven, expert consensus approach in the Sjögren’s International Collaborative Clinical Alliance cohort. Arthritis Care Res (Hoboken) 64:475–487

    Article  CAS  Google Scholar 

  18. 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 36:340–347

    Article  CAS  Google Scholar 

  19. Vitali C, Moutsopoulos HM, Bombardieri S (1994) 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. Ann Rheum Dis 53:637–647

    Article  CAS  Google Scholar 

  20. 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. P92-01 Study Group. Arch Intern Med 159:174–181

    Article  CAS  Google Scholar 

  21. Vivino FB, Carsons SE, Foulks G et al (2016) New Treatment Guidelines for Sjögren’s Disease. Rheum Dis Clin North Am 42:531–551

    Article  Google Scholar 

  22. Wernicke D, Hess H, Gromnica-Ihle E et al (2008) Ultrasonography of salivary glands—a highly specific imaging procedure for diagnosis of Sjögren’s syndrome. J Rheumatol 35:285–293

    PubMed  Google Scholar 

  23. Witte T, Matthias T, Oppermann M et al (2003) Prevalence of antibodies against alpha-fodrin in Sjögren’s syndrome: comparison of 2 sets of classification criteria. J Rheumatol 30:2157–2159

    PubMed  Google Scholar 

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

    Article  Google Scholar 

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Correspondence to Torsten Witte.

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T. Witte gibt an, dass kein Interessenkonflikt besteht.

Für diesen Beitrag wurden vom Autor keine Studien an Menschen oder Tieren durchgeführt. Für die aufgeführten Studien gelten die jeweils dort angegebenen ethischen Richtlinien.

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M. Aringer, Dresden

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Witte, T. Sjögren-Syndrom. Z Rheumatol 78, 511–517 (2019). https://doi.org/10.1007/s00393-019-0625-8

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  • DOI: https://doi.org/10.1007/s00393-019-0625-8

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