Pathophysiology of Sjögren's Syndrome and its diagnostic difficulty

Authors

DOI:

https://doi.org/10.33448/rsd-v10i7.16989

Keywords:

Sjögren's Syndrome; Autoantibodies; Exocrine glands.

Abstract

Sjögren's Syndrome is an autoimmune disease, characterized by lymphocytic infiltration in the glandular epithelium, where it affects mainly the exocrine salivary and lacrimal glands. The study shows the classifications of the syndrome and the relation of autoantibodies related to its diagnosis. An integrative literature review was conducted, using articles published between the years 2010 and 2020, in Portuguese, English and Spanish languages that were published integrally in PubMed, SciELO and LILACS databases. Sicca syndrome is caused by genetic, environmental and hormonal factors. It affects mainly women with an average age of 56 years, where they have been suffering from hormonal imbalance. The autoimmune reaction is caused by the overstimulation of T cells and B cells that will stimulate hypergammaglobulinemia and the production of autoantibodies. In the long term the changes may mutate and lead to the development of non-Hodgkin lymphoma. The literature shows that the delay in diagnosis for Sjögren's Syndrome is due to the subtle symptoms that often go unnoticed or are confused with other pathologies. Anti-Ro and anti-La are the most frequent autoantibodies in patients with SS.

References

Bayetto, K., & Logan, R. M. (2010). Sjögren’s syndrome: a review of aetiology, pathogenesis, diagnosis and management. Australian Dental Journal, 55 Suppl 1, 39–47. https://doi.org/10.1111/j.1834-7819.2010.01197.x

Betim, F., Souza, S., Gomes, R., & Ribeiro, C. (2015). Da Descoberta às Perpectivas da Síndrome de Sjögren: uma Revisão de Literatura. Revista Eletrônica Biociências, Biotecnologia e Saúde, Curitiba, 13, 20–31.

Both, T., Dalm, V. A. S. H., Martin van Hagen, P., & Van Daele, P. L. A. (2017). Reviewing primary Sjögren’s syndrome: Beyond the dryness - From pathophysiology to diagnosis and treatment. International Journal of Medical Sciences, 14(3), 191–200. https://doi.org/10.7150/ijms.17718

Cavalcante, W., Lorenço, S., Florezi, G., Bologna, S., Hsieh, R., Vanini, J., Antunes, M., Paula, F., Pasoto, S., Silva, L., & Nico, M. (2017). Síndrome de Sjögren primária : aspectos relevantes para os dentistas. Clinical and Laboratorial Research in Dentistry, 1–11.

Cornec, D., Devauchelle-Pensec, V., Tobón, G. J., Pers, J. O., Jousse-Joulin, S., & Saraux, A. (2012). B cells in Sjögren’s syndrome: From pathophysiology to diagnosis and treatment. Journal of Autoimmunity, 39(3), 161–167. https://doi.org/10.1016/j.jaut.2012.05.014

COSTA, A. P. DA, JUNIOR, A. C. S., & PINHEIRO, A. L. (2019). Fatores Associados À Etiologia E Patogenese Das Doenças Autoimunes. Arquivos Catarinenses de Medicina, 48(2), 92–104.

Estrela, C. (2018). Metodologia Científica: Ciência, Ensino, Pesquisa. Editora Artes Médicas.

Flament, T., Bigot, A., Chaigne, B., Henique, H., Diot, E., & Marchand-Adam, S. (2016). Pulmonary manifestations of Sjögren’s syndrome. European Respiratory Review, 25(140), 110–123. https://doi.org/10.1183/16000617.0011-2016

Gomes, R., Brandalise, R., Alba, G., Flato, U., & Júnior, J. (2010). Síndrome de Sjögren primária. Revista Da Sociedade Brasileira de Clínica Médica, 8(3), 254–265.

Hajiabbasi, A., Masooleh, I. S., Alizadeh, Y., Banikarimi, A. S., & Ghavidel Parsa, P. (2016). Secondary sjogren’s syndrome in 83 patients with rheumatoid arthritis. Acta Medica Iranica, 54(7), 448–453.

Huang, Y. F., Cheng, Q., Jiang, C. M., An, S., Xiao, L., Gou, Y. C., Yu, W. J., Lei, L., Chen, Q. M., Wang, Y., & Wang, J. (2013). The immune factors involved in the pathogenesis, diagnosis, and treatment of Sjogren’s syndrome. Clinical and Developmental Immunology, 2013. https://doi.org/10.1155/2013/160491

Jonsson, R., Brokstad, K. A., Jonsson, M. V., Delaleu, N., & Skarstein, K. (2018). Current concepts on Sjögren’s syndrome – classification criteria and biomarkers. European Journal of Oral Sciences, 126, 37–48. https://doi.org/10.1111/eos.12536

Kollert, F., & Fisher, B. A. (2020). Equal rights in autoimmunity: Is Sjögren’s syndrome ever “secondary”? Rheumatology (United Kingdom), 59(6), 1218–1225. https://doi.org/10.1093/rheumatology/keaa009

Kyriakidis, N. C., Kapsogeorgou, E. K., & Tzioufas, A. G. (2014). A comprehensive review of autoantibodies in primary Sjögren’s syndrome: Clinical phenotypes and regulatory mechanisms. Journal of Autoimmunity, 51, 67–74. https://doi.org/10.1016/j.jaut.2013.11.001

Lieberman, S. M. (2013). Childhood Sjögren syndrome: Insights from adults and animal models. Current Opinion in Rheumatology, 25(5), 651–657. https://doi.org/10.1097/BOR.0b013e328363ed23

Molina, G., Alegre, G., & Peregrina, M. (2011). The meaning of anti-Ro and anti-La antibodies in primary Sjögren’s syndrome. Autoimmunity Reviews, 10(3), 123–125. https://doi.org/10.1016/j.autrev.2010.09.001

Nisihara, R., Skare, T. L., Cenci, E., Gabardo, D., Nass, F., & Utiyama, S. R. R. (2016). Anti-alpha-fodrin antibodies in patients with Sjögren’s syndrome secondary to rheumatoid arthritis. Jornal Brasileiro de Patologia e Medicina Laboratorial, 52(3), 137–141. https://doi.org/10.5935/1676-2444.20160023

Pasoto, S. G., Martins, V. A. de O., & Bonfa, E. (2019). Sjögren’s syndrome and systemic lupus erythematosus: Links and risks. Open Access Rheumatology: Research and Reviews, 11, 33–45. https://doi.org/10.2147/OARRR.S167783

Santos, A., Bruno, M., Sandoval, P., Albanesi, R., Flores, R., Zanine, R., & Zanini, R. (2013). SÍNDROME DE SJÖGREN. Journal of Biodentistry and Biomaterials - Universidade Ibirapuera São Paulo, 3, 47–54.

Santos, L., Barbalho, J., Bartoli, M., Amaral, M., & Vasconcelos, B. (2013). Síndrome de Sjogren Primária - relato de caso. Cir. Traumatol. Buco-Maxilo-Fac, 13(2), 63–68.

Stefanski, A. L., Tomiak, C., Pleyer, U., Dietrich, T., Burmester, G. R., & Dörner, T. (2017). The diagnosis and treatment of Sjögren’s syndrome. Deutsches Arzteblatt International, 114(20). https://doi.org/10.3238/arztebl.2017.0354

Tsukamoto, M., Suzuki, K., & Takeuchi, T. (2018). Ten-year observation of patients with primary Sjögren’s syndrome: Initial presenting characteristics and the associated outcomes. International Journal of Rheumatic Diseases, 22(5), 929–933. https://doi.org/10.1111/1756-185X.13464

Zerón, P. B., Baldini, C., Bootsma, H., Bowman, S. J., Jonsson, R., Mariette, X., Sivils, K., Theander, E., Tzioufas, A., & Ramos-Casals, M. (2016). Sjögren syndrome. Nature Reviews Disease Primers, 2(July), 1–20. https://doi.org/10.1038/nrdp.2016.47

Zerón, P. B., & Casals, M. R. (2014). Advances in the understanding and treatment of systemic complications in Sjögren’s syndrome. Current Opinion in Rheumatology, 26(5), 520–527. https://doi.org/10.1097/BOR.0000000000000096

Published

26/06/2021

How to Cite

ARAÚJO, C. V. de; SILVA, N. M.; SOUZA, P. G. V. D. de. Pathophysiology of Sjögren’s Syndrome and its diagnostic difficulty. Research, Society and Development, [S. l.], v. 10, n. 7, p. e41010716989, 2021. DOI: 10.33448/rsd-v10i7.16989. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/16989. Acesso em: 13 dec. 2024.

Issue

Section

Review Article