Primary Sjögren’s Syndrome
Authors:
M. Šembera; R. Slezák; V. Radochová
Authors‘ workplace:
Stomatologická klinika LF UK a FN, Hradec Králové
Published in:
Česká stomatologie / Praktické zubní lékařství, ročník 118, 2018, 2, s. 39-47
Category:
Review Article
Overview
Background:
Primary Sjögren‘s syndrome is a progressive autoimmune disease that affects predominantly middle-aged women, typically in the fourth to sixth decenium. It is believed the third most common autoimmune disease after rheumatoid arthritis and systemic lupus erythematosus. The cause of the disease is not fully recognized but a combination of inherited disposition, influence of sex hormones, infectious and environmental factors has been assumed. The examination algorithm requires ophthalmological, dental and immuno-rheumatological examinations as per current diagnostic criteria are from 2016. Despite of all advances in diagnostic methods, the diagnosis of the primary Sjögren‘s syndrome remains rather difficult because of the variability of clinical manifestations. The disease manifests most often by xerostomia and xerophthalmia caused by a lymphocytic infiltration of the salivary and lacrimal glands. In addition, patients often suffer from various musculoskeletal, cutaneous, internal and gynecologic disorders, as well. Clinical manifestations of Sjögren‘s syndrome in the oral cavity are usually the initial symptoms. Oral signs may lead to the suspicion for the disease.
Conclusion:
The review deals with history, epidemiology, etiology and pathogenesis, immunopathology, diagnostics, clinical symptoms and therapy of the primary Sjögren‘s syndrome.
Keywords:
primary Sjögren‘s syndrome – sicca syndrome – xerostomia – malignant lymphoma – immunopathology – autoantibodies – salivary glands – lacrimal glands
Sources
1. Aoun, G., Nasseh, I., Berbe, A.: Evaluation of the oral component of Sjögren‘s syndrome: An overview. J. Int. Soc. Prev. Community Dent., roč. 6, 2016, č. 4, s. 278–284.
2. Ballester, C., Grobost, V., Roblot, P., Pourrat, O., Pierre, F., Laurichesse-Delmas, H., Gallot, D., Aubard, Y., Bezanahary, H., Fauchais, A. L.: Pregnancy and primary Sjögren‘s syndrome: management and outcomes in a multicentre retrospective study of 54 pregnancies. Scand. J. Rheumatol., roč. 46, 2017, č. 1, s. 56–63.
3. Delaleu, N., Jonsson, R., Koller, M. M.: Sjögren‘s syndrome. Eur. J. Oral. Sci., roč. 113, 2005, č. 2, s. 101–113.
4. Ebert, E. C.: Gastrointestinal and hepatic manifestations of Sjőgren syndrome. J. Clin. Gastroenterol., roč. 46, 2012, č. 1, s. 25–30.
5. Drahošová, M., Hrnčíř, Z., Andrýs, C., Slezák, R., Havlasová, J., Kopecký, O.: Analýza autoprotilátky proti 52 kDa a 60 kDa Ro/SSA u pacientek se Sjőgrenovým syndromem a systémovým lupus erythematodes imunoblotem. Rheumatologie, XIV, 2000, č. 1, s. 1–6.
6. Fauchais, A. L., Ouattara, B., Gondran, G., Lalloué, F., Petit, D., Ly, K., Lambert, M., Launay, D., Loustaud-Ratti, V., Bezanahari, H., Liozon, E., Hachulla, E., Jauberteau, M. O., Vidal, E., Hatron, P. Y.: Articular manifestations in primary Sjögren‘s syndrome: clinical significance and prognosis of 188 patients. Rheumatology (Oxford)., roč. 49, 2010, č. 6, s 1164–1172.
7. Flament, T., Bigot, A., Chaigne, B., Henique, H., Diot, E., Marchand-Adam, S.: Pulmonary manifestations of Sjögren‘s syndrome. Eur. Respir. Rev., roč. 25, 2016, č. 140, s. 110–123.
8. Foulks, G. N., Forstot, S. L., Donshik, P. C., Forstot, J. Z., Goldstein, M. H., Lemp, M. A., Nelson, J. D., Nichols, K. K., Pflugfelder, S. C., Tanzer, J. M., Asbell, P., Hammitt, K., Jacobs, D. S.: Clinical guidelines for management of dry eye associated with Sjögren disease. Ocul. Surf., roč. 13, 2015, č. 2, s. 118–132.
9. Fragkioudaki, S., Mavragani, C. P., Moutsopoulos, H. M.: Predicting the risk for lymphoma development in Sjogren syndrome: An easy tool for clinical use. Medicine (Baltimore), roč. 95, 2016, č. 25, s. e3766.
10. François, H., Mariette, X.: Renal involvement in primary Sjögren syndrome. Nat. Rev. Nephrol., roč. 12, 2016, č. 2, s. 82–93.
11. González, S., Sung, H., Sepúlveda, D., González, M., Molina, C.: Oral manifestations and their treatment in Sjögren‘s syndrome. Oral Dis., roč. 20, 2014, č. 2, s. 153–161.
12. Gougerot, H.: Insuffisance progressive et atrophie des glandes salivaires et muqueuses de la bouche, des conjonctives (et parfois des muqueuses nalase, laryngee, vulvaire). Sécheresse de la bouche, des conjonctives, etc. Bull. Med. (Paris), roč. 40, 1926, s. 360–368.
13. Hadden, W. B.: On dry mouth, or suppression of the salivary and buccal secretions. Transactions of the Clinical Society of London. Vol 5. London, England Longmans Green & Co, 1888, s. 176–179.
14. Haga, H. J., Gjesdal, C. G., Koksvik, H. S., Skomsvoll, J. F., Irgens, L. M., Ostensen, M.: Pregnancy outcome in patients with primary Sjögren‘s syndrome. A case-control study. J. Rheumatol., roč. 32, 2005, č. 9, s. 1734–1736.
15. Harboe, E., Tjensvoll, A. B., Vefring, H. K., Gøransson, L. G., Kvaløy, J. T., Omdal, R.: Fatigue in primary Sjögren‘s syndrome – a link to sickness behaviour in animals? Brain Behav. Immun., roč. 23, 2009, č. 8, s. 1104–1108.
16. Hatron, P. Y., Tillie-Leblond, I., Launay, D., Hachulla, E., Fauchais, A. L., Wallaert, B.: Pulmonary manifestations of Sjögren‘s syndrome. Presse Med., roč. 40, 2011, č. 1, s. e49–64.
17. Hejcmanová, D., Němcová, I., Slezák, R.: Podrobné zhodnocení zrakových funkcí pacientů se Sjőgrenovým syndromem před a po zavedení intrakanalikulárních implantátů (typ Smart Plug) (první výsledky). Čes.-slov. Oftalmol., roč. 62, 2006, č. 3, s. 183–189.
18. Chai, J., Logigian, E. L.: Neurological manifestations of primary Sjogren‘s syndrome. Current Opin. Neurol., roč. 23, 2010, č. 5, s. 509–513.
19. Jean, S., Quelvennec, E., Alizadeh, M., Guggenbuhl, P., Birebent, B., Perdriger, A., Grosbois, B., Pawlotsky, P. Y., Semana, G.: DRB1*15 and DRB1*03 extended haplotype interaction in primary Sjögren‘s syndrome genetic susceptibility. Clin. Exp. Rheumatol., roč. 16, 1998, č. 6, s. 725–728.
20. Krejsek, J., Koláčková, M., Lindrová, I., Slezák, R., Andrýs, C.: Increase of intracellular BAFF in B cells of Sjőgren´s patiens is not affected by decrease of BAFFR. Acta Medica (Hradec Králové), roč. 58, 2015, č. 1, s. 25–31.
21. Kong, L., Ogawa, N., Nakabayashi, T., Liu, G. T., D‘Souza, E., McGuff, H. S., Guerrero, D., Talal, N., Dang, H.: FasandFas ligand expression in the salivary glands of patients with primary Sjögren‘s syndrome. Arthritis Rheum., roč. 40, 1997, č. 1, s. 87–97.
22. Mavragani, C. P., Moutsopoulos, H. M.: The geoepidemiology of Sjögren‘s syndrome. Autoimmun. Rev., roč. 9, 2010, č. 5, s. A305–310.
23. Mikulicz-Radecki, J. von.: Über eine eigenartige symmetrische Erkrankung der Thränen- und Mundspeicheldrüsen. In: Beiträge zur Chirurgie. Festschrift gewidmet. Stuttgart: Theodor Billroth; 1892, s. 610–630.
24. Murube, J.: Henrik Sjögren, 1899–1986. The Ocular Surface, roč. 8, č. 1, s. 2–7.
25. Nováková, V., Belada, D., Laco, J., Kašparová, P., Nová, M., Slezák, R.: Malignant lymphomas of non-Hodgkin type in the annals of Stomatology Clinic in Hradec Králové 1998–2008. Čes. Stomatol., roč. 111, 2011, č. 2, s. 36–45.
26. Priori, R., Medda, E., Conti, F., Cassarà, E. A., Sabbadini, M. G., Antonioli, C. M., Gerli, R., Danieli, M. G., Giacomelli, R., Pietrogrande, M., Valesini, G., Stazi, M. A.: Risk factors for Sjögren‘s syndrome: a case-control study. Clin. Exp. Rheumatol., roč. 25, 2007, č. 3, s. 378–384.
27. Qin, B., Wang, J., Yang, Z., Yang, M., Ma, N., Huang, F., Zhong, R.: Epidemiology of primary Sjögren‘s syndrome: a systematic review and meta-analysis. Ann. Rheum. Dis., roč. 74, 2015, č. 11, s. 1983–1989.
28. Rischmueller, M., Tieu, J., Lester, S.: Primary Sjögren‘s syndrome. Best Pract. Res. Clin. Rheumatol., roč. 30, 2016, č. 1, s. 189–220.
29. Roguedas, A. M., Misery, L., Sassolas, B., Le Masson, G., Pennec, Y. L., Youinou, P.: Cutaneous manifestations of primary Sjögren‘s syndrome are underestimated. Clin. Exp. Rheumatol., roč. 22, 2004, č. 5, s. 632–636.
30.Royer, B., Cazals-Hatem, D., Sibilia, J., Agbalika, F., Cayuela, J. M., Soussi, T., Maloisel, F., Clauvel, J. P., Brouet, J. C., Mariette, X.: Lymphomas in patients with Sjogren‘s syndrome are marginal zone B-cell neoplasms, arise in diverse extranodal and nodal sites, and are not associated with viruses. Blood, roč. 90, 1997, č. 2, s. 766–775.
31. Saied, F., Włodkowska-Korytkowska, M., Maślińska, M., Kwiatkowska, B., Kunisz, W., Smorawińska, P., Sudoł-Szopińska, I.: The usefulness of ultrasound in the diagnostics of Sjögren‘s syndrome. J. Ultrason., roč. 13, 2013, č. 53, s. 202–211.
32. Shiboski, C. H., Shiboski, S. C., Seror, R., Criswell, L. A., Labetoulle, M., Lietman, T. M., Rasmussen, A., Scofield, H., Vitali, C., Bowman, S. J., Mariette, X. and the International Sjogren‘s Syndrome Criteria Working Group.: 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary Sjogren‘s Syndrome. Arthritis Rheumatol., roč. 00, 2016, č. 00, s. 00–00.
33. Sjögren, H.: Zur Kenntnis der Keratoconjunctivitis Sicca Keratitis filiformis bei hypofunction der Tränendrüsen. Acta Ophthalmol. (Suppl. II), 1933, s. 1–151.
34. Skálová, S., Minxová, L., Slezák, R.: Hypokalemic paralysis revealing Sjőgren´s syndrome in a 16-year-old girl. Ghana Med. J., roč. 42, 2008, č. 3, s. 124–128.
35. Slezák, R., Rencová, E., Krejsek, J.: Sjögrenův syndrom – polysystémové onemocnění. Pohled stomatologa, oftalmologa a klinického imunologa. Folia Gastroenterol. Hepatol., roč. 1, 2003, č. 1, s. 30–47.
36. Szodoray, P., Barta, Z., Lakos, G., Szakáll, S., Zeher, M.: Coeliac disease in Sjögren‘s syndrome – a study of 111 Hungarian patients. Rheumatol. International, roč. 24, 2004, č. 5, s. 278–282.
37. Teixeira Mendes, L. S., Wotherspoon, A.: Marginal zone lymphoma: Associated autoimmunity and auto-immune disorders. Best Pract. Res. Clin. Haematol., roč. 30, 2017, č.1–2, s. 65–76.
38. Vitali, C., Bombardieri, S., Jonsson, R., Moutsopoulos, H. M., Alexander, E. L., Carsons, S. E., Daniels, T. E., Fox, P. C., Fox, R. I., Kassan, S. S., Pillemer, S. R., Talal, N., Weisman, M. H; European Study Group on Classification Criteria for Sjögren‘s Syndrome.: Classification criteria for Sjögren‘s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann. Rheum. Dis., roč. 61, 2002, č. 6, s. 554–558.
39. Voulgarelis, M., Moutsopoulos, H. M.: Mucosa-associated lymphoid tissue lymphoma in Sjögren‘s syndrome: risks, ma-nagement, and prognosis. Rheum. Dis. Clin. North Am., roč. 34, 2008, č.4, s. 921–933.
40. Voulgarelis, M.,Tzioufas, A. G.: Pathogenetic mechanisms in the initiation and perpetuation of Sjögren‘s syndrome. Nat. Rev. Rheumatol., roč. 6, 2010, č. 9, s. 529–537.
41. Yonetsu, K., Takagi, Y., Sumi, M., Nakamura, T., Eguchi, K.: Sonography as a replacement for sialography for the diagnosis of salivary glands affected by Sjögren‘s syndrome. Ann. Rheum. Dis., roč. 61, 2002, č. 3, s. 276–277.
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Maxillofacial surgery Orthodontics Dental medicineArticle was published in
Czech Dental Journal
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