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© 2025. Springer Nature Limited. Nomenclature for the disease widely known as Sjögren syndrome has proven unsatisfactory. Patients have perceived 'syndrome' as indicative of a vague collection of symptoms, prompting the Sjögren's Foundation to abandon the term. Furthermore, the traditional distinction between 'primary' and 'secondary' forms fails to account for the complex interplay between overlapping autoimmune diseases. Following a bibliometric analysis, systematic literature review and a Delphi consensus process with equal involvement of professional and patient representatives, five recommendations are now issued. First, the term 'Sjögren disease' should replace 'Sjögren syndrome'. Second, the acronym 'SjD' should be used as an abbreviation for 'Sjögren disease'. Third, the descriptor 'associated' should be used in lieu of 'secondary' for Sjögren disease occurring in association with a second systemic autoimmune disease for which classification criteria are fulfilled. Fourth, Sjögren disease is the preferred terminology in common parlance and in clinical diagnosis, without differentiation as to primary and associated forms. Fifth, the differentiation between primary and associated Sjögren is recommended for scientific studies to define a homogeneous population. In conclusion, the consensus endorses 'Sjögren disease' as the official nomenclature to acknowledge the distinct pathogenesis of this disorder and to improve clarity in both clinical practice and research.
Author(s): Ramos-Casals M, Baer AN, Brito-Zeron MDP, Hammitt KM, Bouillot C, Retamozo S, Mackey A, Yarowsky D, Turner B, Blanck J, Fisher BA, Akpek EK, Baldini C, Bootsma H, Bowman SJ, Dorner T, Laing L, Lieberman SM, Mariette X, Pflugfelder SC, Sankar V, Siso-Almirall A, Tzioufas AG, Anaya J-M, Armagan B, Bombardieri M, Carsons S, de Vita S, Fox RI, Gerli R, Giacomelli R, Gottenberg JE, Hernandez-Molina G, Jonsson R, Kruize A, Kwok S-K, Li X, McCoy SS, Ng W-F, Olsson P, Rischmueller M, Saraux A, Scofield RH, Valim V, Vitali C, Vivino F, Wahren-Herlenius M, Moutsopoulos HM
Publication type: Review
Publication status: Published
Journal: Nature Reviews Rheumatology
Year: 2025
Volume: 21
Issue: 7
Pages: 426-437
Print publication date: 01/07/2025
Online publication date: 10/06/2025
Acceptance date: 08/05/2025
ISSN (print): 1759-4790
ISSN (electronic): 1759-4804
URL: https://doi.org/10.1038/s41584-025-01268-z
DOI: 10.1038/s41584-025-01268-z
PubMed id: 40494962
