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Epidemiology of Sjögren syndrome

Nature Reviews Rheumatology volume 20, pages 158–169 (2024)Cite this article

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Abstract

Sjögren syndrome is a phenotypically varied autoimmune disorder that can occur alone in primary Sjögren syndrome or in association with other connective tissue diseases (CTDs), including rheumatoid arthritis, systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). The estimation of the prevalence and incidence of Sjögren syndrome varies depending on diagnostic criteria and study design, making it difficult to estimate geographical and temporal trends. Nonetheless, disease phenotype is influenced by geographical origin, which is a risk factor for systemic activity. Whether mortality in primary Sjögren syndrome is increased compared with that of the general population is not yet known, but extra-glandular manifestations, in particular lymphomas, are clear risk factors for mortality. In CTDs associated with Sjögren syndrome, lymphoma risk seems higher than that of patients with CTD alone, and there is potentially lower disease activity in SLE with Sjögren syndrome and in SSc with Sjögren syndrome than in SLE or SSc alone.

Key points

  • Primary Sjögren syndrome (pSS) affects mostly women, with age at disease onset as well as systemic activity varying according to geographical origin.

  • Assessment of the prevalence of pSS varies according to study methodology, but can be estimated at between 0.01% and 0.05%, although some countries might be under-represented in this estimation.

  • Mortality in pSS seems to be elevated predominantly in patients with extra-glandular manifestations, pulmonary involvement and non-Hodgkin lymphoma.

  • pSS is the autoimmune disease with the most important increased risk of non-Hodgkin lymphoma, especially among patients with extra-glandular manifestations or systemic activity, low complement levels, cryoglobulinaemic vasculitis or rheumatoid factor positivity.

  • Sjögren syndrome can be associated with other connective tissue diseases, mainly rheumatoid arthritis, systemic lupus erythematosus and systemic sclerosis.

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Fig. 1: Mapped prevalence of primary Sjögren syndrome.

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Authors and Affiliations

  1. Department of Rheumatology, Bicêtre AP-HP Hôpital, Université Paris-Saclay, Paris, France

    Maxime Beydon, Yann Nguyen, Xavier Mariette & Raphaèle Seror

  2. Department of Medicine, University of Wisconsin-Madison, Madison, Wisconsin, USA

    Sara McCoy

  3. Center for Immunology of Viral Infections and Auto-Immune Diseases, INSERM U1184, Université Paris Saclay, Le Kremlin-Bicêtre, France

    Yann Nguyen, Xavier Mariette & Raphaèle Seror

  4. Department of Internal Medicine, University of Tsukuba, Tsukuba, Japan

    Takayuki Sumida

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Contributions

M.B., S.M., Y.N., X.M. and R.S. researched data for the article. All authors made substantial contributions to discussion of the content. M.B., S.M, Y.N., X.M. and R.S. wrote the article. All authors reviewed and/or edited the manuscript before submission.

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Correspondence to Raphaèle Seror.

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Competing interests

S.M. has received consulting fees from Bristol Myers Squibb, Horizon, iCell, Kiniksa, Novartis, Otsuka, Target RWE and Visterra. X.M. has received consulting fees from Astra Zeneca, Bristol Myers Squibb, Galapagos, GSK, Novartis and Pfizer. R.S. has received consulting fees from Amgen, Bristol Myers Squibb, Boehringer, GSK, Janssen and Pfizer, and travel fees from Amgen and GSK. The remaining authors declare no competing interests.

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Beydon, M., McCoy, S., Nguyen, Y. et al. Epidemiology of Sjögren syndrome. Nat Rev Rheumatol 20, 158–169 (2024). https://doi.org/10.1038/s41584-023-01057-6

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