Publication

2023 International Rome consensus for the nomenclature of Sjögren disease

Abstract
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
Ramos-Casals, Manuel
Baer, Alan N
Brito-Zerón, María Del Pilar
Hammitt, Katherine M
Bouillot, Coralie
Retamozo, Soledad
Mackey, Alison
Yarowsky, David
Turner, Breck
Blanck, Jaime
Fisher, Benjamin A
Akpek, Esen K
Baldini, Chiara
Bootsma, Hendrika
Bowman, Simon J
Dörner, Thomas
Laing, Leslie
Lieberman, Scott M
Mariette, Xavier
Pflugfelder, Stephen C
Sankar, Vidya
Sisó-Almirall, Antoni
Tzioufas, Athanasios G
Anaya, Juan-Manuel
Armağan, Berkan
Bombardieri, Michele
Carsons, Steven
de Vita, Salvatore
Fox, Robert I
Gerli, Roberto
Giacomelli, Roberto
Gottenberg, Jacques Eric
Hernández-Molina, Gabriela
Jonsson, Roland
Kruize, Aike
Kwok, Seung-Ki
Li, Xiaomei
McCoy, Sara S
Ng, Wan-Fai
Olsson, Peter
Rischmueller, Maureen
Saraux, Alain
Scofield, R Hal
Valim, Valéria
Vitali, Claudio
Vivino, Frederick
Wahren-Herlenius, Marie
Moutsopoulos, Haralampos M
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Date
2025-06-10
Type
Article
Subject
Allergy and Immunology, Rheumatology, Ophthalmology
Collections
UHB Allergy and Immunology
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Citation
Ramos-Casals M, Baer AN, Brito-Zerón 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, Dörner T, Laing L, Lieberman SM, Mariette X, Pflugfelder SC, Sankar V, Sisó-Almirall A, Tzioufas AG, Anaya JM, Armağan B, Bombardieri M, Carsons S, de Vita S, Fox RI, Gerli R, Giacomelli R, Gottenberg JE, Hernández-Molina G, Jonsson R, Kruize A, Kwok SK, Li X, McCoy SS, Ng WF, Olsson P, Rischmueller M, Saraux A, Scofield RH, Valim V, Vitali C, Vivino F, Wahren-Herlenius M, Moutsopoulos HM; International Task Force on Nomenclature of Sjögren Disease. 2023 International Rome consensus for the nomenclature of Sjögren disease. Nat Rev Rheumatol. 2025 Jul;21(7):426-437. doi: 10.1038/s41584-025-01268-z. Epub 2025 Jun 10.
Journal / Source Title
Nature reviews. Rheumatology
DOI
10.1038/s41584-025-01268-z
URI
https://mider.dspace7.openrepository.com/handle/20.500.14200/8478
PMID
40494962
Publisher
Nature
Publisher’s URL
http://www.nature.com/nrrheum
Publisher’s statement
Note / Copyright