The Prevalence of Sjögren's syndrome in different geographic regions: a review of the literature

uma revisão da literatura

Abstract

Introduction: Sjögren's Syndrome (SS) is a chronic inflammatory autoimmune disease, characterized by lymphocytic infiltrate in the epithelium and exocrine glands, affecting mainly the salivary and lacrimal glands, resulting in a decrease in their fluids¹. In SS, several clinical manifestations can be observed, such as xerostomia, frequent cavities, mouth ulcers and ulcers on the lip mucosa, pain when swallowing, oral infections and xerophthalmia²^,³. This disease is described in the current literature in two forms: Primary Sjögren's Syndrome (pSS), when it involves only the exocrine glands, and secondary Sjögren's Syndrome (SSs), when it is related to other autoimmune pathologies, such as lúpus systemic erythematosus, rheumatoid arthritis and systemic sclerosis¹. It is worth mentioning that there is no cure for SS, with treatment being only palliative, with the aim of minimizing the patient's discomfort and delaying the progression of the disease⁴. Furthermore, this pathology is relatively common, but there are few studies on it is prevalence by geographic region in the world. The results regarding the prevalence of SS contribute to the elucidation of the profile of this disease, it is impact on society and open paths for a better understanding of it, which makes it conceivable to create public policies that meet the demands caused by the disease⁵.

Objective: To identify, through a bibliographic search, the prevalence of primary Sjögren's Syndrome (pSS) and secondary Sjögren's Syndrome (SSs) in different geographic regions of the world.

Methodology: A bibliographic search was carried out from November 2019 to April 2023 in the databases: PubMed, SciELO, ScienceDirect, Lilacs, Web of Science and also in gray literature, through Google Scholar. Articles published in the last 20 years were included, without language restrictions, with a selection of those that presented the content of interest and were available in full. Studies such as literature reviews, systematic reviews, clinical case reports and book chapters were excluded from the research, along with studies whose samples were restricted to patients diagnosed with SS, not presenting a real prevalence value for this disease.

Results: 31 articles were included, covering descriptive and analytical observational epidemiological studies: cross-sectional, demographic, prospective and retrospective cohort studies, as well as longitudinal. Research was found relating to the continents of America, Europe and Asia. In America, 13 articles were selected, and prevalence values ​​for pSS ranged from 0.0049% to 21.7%, while for SSs it ranged from 4.3% to 24.39%. In Europe, for SSp, a variation of 0.01% to 12.55% was found, and for SSs, 3.6% to 14.28%, based on a total of 12 studies. In Asia, 6 articles were found, and for pSS, the prevalence ranged from 0.016% to 1.56%, while for SSs it ranged from 5.5% to 35.3%.

Conclusion: The prevalence of Sjögren's Syndrome varied in all regions of the world, due to different classification criteria, sample diversity and lack of standardization of the methodologies adopted, which resulted in a disparity in values. Furthermore, the lack of classification criteria that include SSs was also a factor that influenced bias in the research.