Pathophysiology of Sjögren's Syndrome and its diagnostic difficulty
DOI:
https://doi.org/10.33448/rsd-v10i7.16989Keywords:
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
Downloads
Published
How to Cite
Issue
Section
License
Copyright (c) 2021 Cassia Vilar de Araújo; Natália Millena Silva; Pâmella Grasielle Vital Dias de Souza
This work is licensed under a Creative Commons Attribution 4.0 International License.
Authors who publish with this journal agree to the following terms:
1) Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
2) Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
3) Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.