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2023 International Rome consensus for the nomenclature of Sjögren disease

Ramos-Casals, Manuel ; Baer, Alan N. ; Brito-Zerón, María del Pilar ; Hammitt, Katherine M. ; Bouillot, Coralie ; Retamozo, Soledad ; Mackey, Alison ; Yarowsky, David ; Turner, Breck and Blanck, Jaime , et al. (2025) In Nature Reviews Rheumatology 21(7). p.426-437
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’.... (More)

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.

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type
Contribution to journal
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published
subject
in
Nature Reviews Rheumatology
volume
21
issue
7
pages
12 pages
publisher
Nature Publishing Group
external identifiers
  • scopus:105009928363
  • pmid:40494962
ISSN
1759-4790
DOI
10.1038/s41584-025-01268-z
language
English
LU publication?
yes
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Publisher Copyright: © Springer Nature Limited 2025.
id
708ce438-8d9a-46a5-9633-ae6a4ef7c306
date added to LUP
2025-12-15 13:19:24
date last changed
2025-12-16 09:38:38
@article{708ce438-8d9a-46a5-9633-ae6a4ef7c306,
  abstract     = {{<p>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.</p>}},
  author       = {{Ramos-Casals, Manuel and Baer, Alan N. and Brito-Zerón, María del Pilar and Hammitt, Katherine M. and Bouillot, Coralie and Retamozo, Soledad and Mackey, Alison and Yarowsky, David and Turner, Breck and Blanck, Jaime and Fisher, Benjamin A. and Akpek, Esen K. and Baldini, Chiara and Bootsma, Hendrika and Bowman, Simon J. and Dörner, Thomas and Laing, Leslie and Lieberman, Scott M. and Mariette, Xavier and Pflugfelder, Stephen C. and Sankar, Vidya and Sisó-Almirall, Antoni and Tzioufas, Athanasios G. and Anaya, Juan Manuel and Armağan, Berkan and Bombardieri, Michele and Carsons, Steven and de Vita, Salvatore and Fox, Robert I. and Gerli, Roberto and Giacomelli, Roberto and Gottenberg, Jacques Eric and Hernández-Molina, Gabriela and Jonsson, Roland and Kruize, Aike and Kwok, Seung Ki and Li, Xiaomei and McCoy, Sara S. and Ng, Wan Fai and Olsson, Peter and Rischmueller, Maureen and Saraux, Alain and Scofield, R. Hal and Valim, Valéria and Vitali, Claudio and Vivino, Frederick and Wahren-Herlenius, Marie and Moutsopoulos, Haralampos M. and Vissink, Arjan and Trevisani, Virginia Fernandes Moça}},
  issn         = {{1759-4790}},
  language     = {{eng}},
  number       = {{7}},
  pages        = {{426--437}},
  publisher    = {{Nature Publishing Group}},
  series       = {{Nature Reviews Rheumatology}},
  title        = {{2023 International Rome consensus for the nomenclature of Sjögren disease}},
  url          = {{http://dx.doi.org/10.1038/s41584-025-01268-z}},
  doi          = {{10.1038/s41584-025-01268-z}},
  volume       = {{21}},
  year         = {{2025}},
}