SJÖGREN’S SYNDROME: PATHOGENESIS, DIAGNOSIS, MANIFESTATIONS, AND MODERN TREATMENT – A NARRATIVE REVIEW
DOI:
https://doi.org/10.31435/ijitss.2(50).2026.5597Keywords:
Sjögren’s Syndrome, Xerophthalmia, Xerostomia, Ro/SSA Antibody, La/SSB Antibody, Pulmonary Fibrosis, TreatmentAbstract
Sjögren's syndrome is a chronic, systemic autoimmune disease whose symptoms affect multiple organs and systems. However, a characteristic feature of this syndrome is exocrine gland dysfunction, manifesting as dry eye and dry mouth. Other typical symptoms include fatigue, musculoskeletal pain, and systemic manifestations such as cutaneous vasculitis, interstitial lung disease, and neuropathy. Sjögren's syndrome, as with other rheumatoid diseases, is more common in women than in men, most often in middle age [1]. Patients with this syndrome also have an increased cardiovascular risk and an increased risk of non-Hodgkin's lymphoma. The first case with dry eye symptoms was described in 1892 by Jan Mikulicz-Radecki, a Polish-German surgeon [2]. We differentiate between primary Sjögren's syndrome and the secondary form associated with other autoimmune diseases, such as rheumatoid arthritis. More than half of patients demonstrate elevated levels of antinuclear antibodies (Ro). The etiology of Sjogren's syndrome remains poorly understood, but several genetic, epigenetic, and environmental factors have been proposed. Diagnosis is based on symptoms of dry eye syndrome, the presence of characteristic autoantibodies, and morphological findings from a minor salivary gland biopsy. Treatment of Sjogren's syndrome is based on symptomatic therapies for dry eyes and the oral cavity. This review also focuses primarily on modern biologic medications and new, promising therapeutic options.
References
Mavragani, C. P., & Moutsopoulos, H. M. (2010). The geoepidemiology of Sjögren’s syndrome. Autoimmunity Reviews, 9(5), A305–A310. https://doi.org/10.1016/j.autrev.2009.11.004
von Mikulicz, J. H. (1892). Über eine eigenartige symmetrische Erkrankung der Tränen- und Mundspeicheldrüsen. Beiträge zur Chirurgie: Festschrift gewidmet Theodor Billroth, 610–630.
Brito-Zerón, P., Acar-Denizli, N., Zeher, M., et al. (2017). Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren’s syndrome at diagnosis in 8310 patients: A cross-sectional study from the Big Data Sjögren Project Consortium. Annals of the Rheumatic Diseases, 76(6), 1042–1050. https://doi.org/10.1136/annrheumdis-2016-209952
Singh, A. G., Singh, S., & Matteson, E. L. (2016). Rate, risk factors, and causes of mortality in patients with Sjögren’s syndrome: A systematic review and meta-analysis of cohort studies. Rheumatology, 55(3), 450–460. https://doi.org/10.1093/rheumatology/kev354
Lessard, C. J., Li, H., Adrianto, I., et al. (2013). Variants at multiple loci implicated in both innate and adaptive immune responses are associated with Sjögren’s syndrome. Nature Genetics, 45(11), 1284–1292. https://doi.org/10.1038/ng.2792
Chivasso, C., Sarrand, J., Perret, J., Delporte, C., & Soyfoo, M. S. (2021). The involvement of innate and adaptive immunity in the initiation and perpetuation of Sjögren’s syndrome. International Journal of Molecular Sciences, 22(2), Article 658. https://doi.org/10.3390/ijms22020658
Ou, T. T., Lin, C. H., Lin, Y. C., et al. (2008). IκBα promoter polymorphisms in patients with primary Sjögren’s syndrome. Journal of Clinical Immunology, 28(5), 440–444. https://doi.org/10.1007/s10875-008-9212-5
Retamozo, S., Acar-Denizli, N., Rasmussen, A., Horváth, I. F., Baldini, C., Priori, R., et al. (2019). Systemic manifestations of primary Sjögren’s syndrome out of the ESSDAI classification: Prevalence and clinical relevance in a large international, multi-ethnic cohort of patients. Clinical and Experimental Rheumatology, 37(Supplement 118), 97–106.
Chiu, Y. H., Chung, C. H., Lin, K. T., Lin, C. S., Chen, J. H., Chen, H. C., et al. (2017). Predictable biomarkers of developing lymphoma in patients with Sjögren syndrome: A nationwide population-based cohort study. Oncotarget, 8(30), 50098–50108. https://doi.org/10.18632/oncotarget.15100
Voulgarelis, M., Ziakas, P. D., Papageorgiou, A., Baimpa, E., Tzioufas, A. G., & Moutsopoulos, H. M. (2012). Prognosis and outcome of non-Hodgkin lymphoma in primary Sjögren syndrome. Medicine, 91(1), 1–9. https://doi.org/10.1097/MD.0b013e31824125e4
Fauchais, A. L., Ouattara, B., Gondran, G., Lalloué, F., Petit, D., Ly, K., et al. (2010). Articular manifestations in primary Sjögren’s syndrome: Clinical significance and prognosis of 188 patients. Rheumatology, 49(6), 1164–1172. https://doi.org/10.1093/rheumatology/keq047
Francois, H., & Mariette, X. (2020). Renal involvement in Sjögren’s syndrome. Nephrologie & Therapeutique, 16(7), 440–452.
Shiboski, C. H., Shiboski, S. C., Seror, R., et al. (2017). 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 & Rheumatology, 69(1), 35–45. https://doi.org/10.1002/art.39859
Seror, R., Ravaud, P., Mariette, X., et al. (2011). EULAR Sjögren’s syndrome patient-reported index (ESSPRI): Development of a consensus patient index for primary Sjögren’s syndrome. Annals of the Rheumatic Diseases, 70(6), 968–972.
Werth, V., Furie, R., Romero-Díaz, J., Navarra, S., Kalunian, K., van Vollenhoven, R., et al. (2020). BIIB059, a humanized monoclonal antibody targeting BDCA2 on plasmacytoid dendritic cells (pDC), shows dose-related efficacy in the phase 2 LILAC study in patients with active cutaneous lupus erythematosus (CLE) [Conference abstract OP0193]. Annals of the Rheumatic Diseases, 79(Supplement 1), 120–121. https://doi.org/10.1136/annrheumdis-2020-eular.5743
Price, E. J., Rauz, S., Tappuni, A. R., et al. (2017). The British Society for Rheumatology guideline for the management of adults with primary Sjögren’s syndrome. Rheumatology, 56(10), 1643–1647. https://doi.org/10.1093/rheumatology/kex163
U.S. National Library of Medicine. (n.d.). ClinicalTrials.gov. https://clinicaltrials.gov/
Brinkmann, V., Billich, A., Baumruker, T., Heining, P., Schmouder, R., Francis, G., et al. (2010). Fingolimod (FTY720): Discovery and development of an oral drug to treat multiple sclerosis. Nature Reviews Drug Discovery, 9, 883–897. https://doi.org/10.1038/nrd3248
Cohen, J. A., Arnold, D. L., Comi, G., Bar-Or, A., Gujrathi, S., Hartung, J. P., et al. (2016). Safety and efficacy of the selective sphingosine 1-phosphate receptor modulator ozanimod in relapsing multiple sclerosis (RADIANCE): A randomised, placebo-controlled, phase 2 trial. The Lancet Neurology, 15(4), 373–381. https://doi.org/10.1016/S1474-4422(16)00018-1
Wu, P., Nielsen, T. E., & Clausen, M. H. (2016). Small-molecule kinase inhibitors: An analysis of FDA-approved drugs. Drug Discovery Today, 21(1), 5–10. https://doi.org/10.1016/j.drudis.2015.07.008
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Copyright (c) 2026 Łukasz Jaworek, Gabriela Zimka, Krystian Bjorgen, Lilianna Jasińska, Magdalena Roman, Martyna Lipiarz, Michał Niespodziewański, Patrycja Szczygielska, Sylwia Hejna, Weronika Smutkiewicz

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