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Sjøgrens syndrom

Sjøgrens syndrom er en betennelse som rammer kroppens kjertler og gir symptomer som tørr munn og tørre øyne. Mange pasienter har i tillegg uklare plager som leddsmerter, muskelsmerter og tretthet.

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Sjøgrens syndrom rammer i 90 prosent av tilfellene kvinner.

Sist oppdatert:

15. sep. 2020

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Hva er Sjøgrens syndrom?

Primært Sjøgrens syndrom er en kronisk betennelse som rammer kroppens kjertler, særlig tårekjertlene og spyttkjertlene. Dette fører til tørre øyne og tørre slimhinner, noe som er de dominerende symptomene. Men så godt som alle organ i kroppen kan rammes av sykdommen og gi et mangfold av symptomer og tegn.

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Sekundært Sjøgrens syndrom er et syndrom som finnes hos en andel av pasientene med revmatiske sykdommer som leddgikt, systemisk lupus erytematosus, sklerodermi og andre autoimmune sykdommer. Også her rammer tørrheten særlig øyne og munn.

Oppdelingen i primært og sekundært Sjøgrens syndrom har ingen betydning for behandlingen - den er den samme.

Over 90 prosent av dem som har primært Sjøgrens syndrom, er kvinner. Sykdommen diagnostiseres oftest i 50-60 års-alderen,  men den kan oppstå i alle aldre, inkludert hos barn. Tall for hvor vanlig sykdommen er, er usikre, men trolig rammer Sjøgrens omtrent 1 av 2000. I aldersgruppen 52-72 år er forekomsten 2,7 prosent. 

Vil du vite mer

Dette dokumentet er basert på det profesjonelle dokumentet Sjøgrens syndrom . Referanselisten for dette dokumentet vises nedenfor

  1. Fox RI. Sjögren's syndrome. Lancet 2005; 366: 321-31. PubMed
  2. Kruszka P, O'Brian RJ. Diagnosis and management of Sjögren syndrome. Am Fam Physician 2009; 79: 465-70. American Family Physician
  3. Kurien BT, Scofield RH. Sjogren's syndrome. BMJ Best Practice, last updated May 23, 2016. bestpractice.bmj.com
  4. Gøransson LG, Haldorsen K, Brun JG,et al. The point prevalence of clinically relevant primary Sjögren's syndrome in two Norwegian counties. Scand J Rheumatol. 2011 May;40(3):221-4. PMID: 21231797. PubMed
  5. Pillemer SR, Matteson EL, Jacobsson LT et al. Incidence of physician-diagnosed primary Sjögren syndrome in residents of Olmsted County. Minnesota, Mayo Clin Proc 2001; 76: 593-9.
  6. Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjogren's syndrome. Nat Rev Rheumatol. 2013;9:544-556. PubMed
  7. Cummins MJ, Papas A, Kammer GM, Fox PC. Treatment of primary Sjögren's syndrome with low-dose human interferon alfa administered by the oromucosal route: combined phase III results. Arthritis Rheum 2003; 49: 585-93. PubMed
  8. Mariette X, Ravaud P, Steinfeld S, et al. Inefficacy of infliximab in primary Sjögren's syndrome: results of the randomized, controlled Trial of Remicade in Primary Sjögren's Syndrome (TRIPSS). Arthritis Rheum 2004; 50: 1270-6. PubMed
  9. 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 Rheum 2004; 50: 882-91. PubMed
  10. Shiboski CH, Shiboski SC, Seror R, et al. 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. Arthritis Rheumatol 2017; 69:35. PubMed
  11. Ramos-Casals M, Anaya JM, Garcia-Carrasco M et al. Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients. Medicine (Baltimore) 2004; 83: 96-106. PubMed
  12. Mariette X, Criswell LA. Primary Sjögren’s Syndrome. N Engl J Med 2018; 378: 931-39. pmid:29514034 PubMed
  13. Lindvall B, Bengtsson A, Ernerudh J and Eriksson P. Subclinical myositis is common in primary Sjögren's syndrome and is not related to muscle pain. J Rheumatol 2002; 29: 717-25. PubMed
  14. Johnsen SJ, Brun JG, Gøransson LG, et al. Risk of non-Hodgkin's lymphoma in primary Sjögren's syndrome: a population-based study. Arthritis Care Res (Hoboken). 2013 May;65(5):816-21. PMID: 23139233. PubMed
  15. Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL and Youinou P. Cutaneous manifestations of primary Sjögren's syndrome are underestimated. Clin Exp Rheumatol 2004; 22: 632-6. PubMed
  16. Chung WS, Lin CL, Sung FC, et al. Increased risks of deep vein thrombosis and pulmonary embolism in Sjogren syndrome: a nationwide cohort study. J Rheumatol. 2014;41:909-915. PubMed
  17. Gamron S, Barberis G, Onetti CM et al. Mesangial nephropathy in Sjögren's syndrome. Scand J Rheumatol 2000; 29; 65-7.
  18. D'Arbonneau F, Ansart S, Le Berre R, Dueymes M, Youinou P and Pennec YL. Thyroid dysfunction in primary Sjögren's syndrome: a long-term followup study. Arthritis Rheum 2003; 49: 804-9. PubMed
  19. Delalande S, de Seze J, Fauchais AL et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine (Baltimore) 2004; 83: 280-91. PubMed
  20. Scofield AK, Radfar L, Ice JA, et al. Relation of sensory peripheral neuropathy in Sjogren syndrome to anti-Ro/SSA. J Clin Rheumatol. 2012;18:290-293. PubMed
  21. Scheinfeld N. Sjogren syndrome and systemic lupus erythematosus are distinct conditions. Dermatol Online J. 2006;12:4. PubMed
  22. Gran JT, Myklebust G. Diagnostikk av primært Sjögrens syndrom. Tidsskr Nor Lægeforen 2001; 121: 563-6. Tidsskrift for Den norske legeforening
  23. Jonsson MV, Baldini C. Major salivary gland ultrasonography in the diagnosis of Sjogren’s syndrome - a place in the diagnostic criteria? Rheum Dis Clin North Am. 2016 Aug;42(3):501-17. PMID: 27431351. PubMed
  24. Vivino FB, Gala I and Hermann GA. Change in final diagnosis on second evaluation of labial minor salivary gland biopsies. J Rheumatol 2002; 29: 938-44. PubMed
  25. Baer AN, Vivino FB. Treatment of Sjögren's syndrome: Constitutional and non-sicca organ-based manifestations. UpToDate, last updated Apr 06, 2020. UpToDate
  26. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren's syndrome. Arch Intern Med 2004; 164: 1275-84. PubMed
  27. Baer AN, Sankar V. Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome. UpToDate, last updated Jul 11, 2020. UpToDate
  28. Belafsky PC and Postma GN. The laryngeal and esophageal manifestations of Sjögren's syndrome. Curr Rheumatol Rep 2003; 5: 297-303. PubMed
  29. Wang SQ, Zhang LW, Wei P, Hua H. Is hydroxychloroquine effective in treating primary Sjogren's syndrome: a systematic review and meta-analysis. BMC Musculoskelet Disord 2017; 18: 186. pmid:28499370 PubMed
  30. Zintzaras E, Voulgarelis M, Moutsopoulos HM. The risk of lymphoma development in autoimmune diseases: a meta-analysis. Arch Intern Med 2005; 165: 2337-44. PubMed
  31. 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. PubMed
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