Journal article
2023 International Rome consensus for the nomenclature of Sjögren disease
Nature reviews. Rheumatology, Vol.21(7), pp.426-437
07/2025
DOI: 10.1038/s41584-025-01268-z
PMID: 40494962
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.
Details
- Title: Subtitle
- 2023 International Rome consensus for the nomenclature of Sjögren disease
- Creators
- Manuel Ramos-Casals - Department of Medicine, University of Barcelona, Barcelona, SpainAlan N Baer - Johns Hopkins University School of MedicineMaría Del Pilar Brito-Zerón - Hospital Sanitas CIMAKatherine M Hammitt - Sjögren’s FoundationCoralie Bouillot - Sjögren Europe, Gland, SwitzerlandSoledad Retamozo - Department of Rheumatology, Hospital Quiron Salud de Barcelona, Barcelona, SpainAlison Mackey - Georgetown UniversityDavid Yarowsky - Johns Hopkins UniversityBreck Turner - Johns Hopkins University School of MedicineJaime Blanck - Johns Hopkins University School of MedicineBenjamin A Fisher - University of BirminghamEsen K Akpek - Johns Hopkins University School of MedicineChiara Baldini - University of PisaHendrika Bootsma - University of GroningenSimon J Bowman - Rheumatology Department, Milton Keynes University Hospital, Milton Keynes, UKThomas Dörner - Charité - Universitätsmedizin BerlinLeslie Laing - Sjögren’s Society of CanadaScott M Lieberman - University of IowaXavier Mariette - Assistance Publique – Hôpitaux de ParisStephen C Pflugfelder - Baylor College of MedicineVidya Sankar - Tufts UniversityAntoni Sisó-Almirall - Primary HealthCareAthanasios G Tzioufas - Biomedical Research Foundation of the Academy of AthensJuan-Manuel Anaya - Universidad de la Costa, Barranquilla, ColombiaBerkan Armağan - Ankara Bilkent City HospitalMichele Bombardieri - Queen Mary University of LondonSteven Carsons - Division of Rheumatology, Allergy and Immunology, Department of Medicine, NYU Grossman Long Island School of Medicine, Mineola, NY, USASalvatore de Vita - University of UdineRobert I Fox - Scripps Memorial HospitalRoberto Gerli - University of PerugiaRoberto Giacomelli - Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Biomedico" School of Medicine, Rome, ItalyJacques Eric Gottenberg - Department of Rheumatology, Centre de Référence National pour les Maladies Autoimmunes Systémiques Rares, IBMC, CNR, UPR 3572, University of Strasbourg, Strasbourg, FranceGabriela Hernández-Molina - Instituto Nacional de Ciencias Médicas y Nutrición Salvador ZubiránRoland Jonsson - University of BergenAike Kruize - University Medical Center UtrechtSeung-Ki Kwok - Catholic University of KoreaXiaomei Li - Department of Rheumatology and Immunology, The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, ChinaSara S McCoy - Department of Medicine, University of Wisconsin School of Medicine and Health, Madison, WI, USAWan-Fai Ng - Newcastle upon Tyne Hospitals NHS Foundation TrustPeter Olsson - Lund UniversityMaureen Rischmueller - The University of AdelaideAlain Saraux - Université de Bretagne OccidentaleR Hal Scofield - Department of Veterans Affairs Medical Center, Oklahoma City, OK, USAValéria Valim - Universidade Federal do Espírito SantoClaudio Vitali - Consultant in Rheumatology, Istituto Clinico 'Mater Domini', Castellanza, ItalyFrederick Vivino - University of PennsylvaniaMarie Wahren-Herlenius - Karolinska InstitutetHaralampos M Moutsopoulos - Academy of AthensInternational Task Force on Nomenclature of Sjögren Disease
- Resource Type
- Journal article
- Publication Details
- Nature reviews. Rheumatology, Vol.21(7), pp.426-437
- DOI
- 10.1038/s41584-025-01268-z
- PMID
- 40494962
- NLM abbreviation
- Nat Rev Rheumatol
- ISSN
- 1759-4804
- eISSN
- 1759-4804
- Publisher
- NATURE PORTFOLIO; BERLIN
- Grant note
- Birmingham NIHR Biomedical Research Centre, Birmingham, UK
These recommendations are endorsed by the Sjogren's Foundation and Sjogren Europe. S.J.B. and B.F. are supported by the Birmingham NIHR Biomedical Research Centre, Birmingham, UK.
- Language
- English
- Electronic publication date
- 06/10/2025
- Date published
- 07/2025
- Academic Unit
- Stead Family Department of Pediatrics; Rheumatology, Allergy, and Immunology
- Record Identifier
- 9984830606802771
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