The Sjögren’s Foundation has been leading the charge to change the name Sjögren’s Syndrome to Sjögren’s Disease, to better reflect the serious and systemic nature of the disease. We started by changing the name of our foundation to the Sjögren’s Foundation in late 2019 – no more “syndrome!”
The process to change the name had several milestones to set the stage and engage the international patient and clinician community to come to consensus.
First Publication in Arthritis and Rheumatology in 2021
After we changed our name, we engaged the broader community in recognizing the need for change by publishing a Letter to the Editor in the American College of Rheumatology’s official journal, Arthritis and Rheumatology: Baer AN and Hammitt KM. Sjögren’s Disease, Not Syndrome. Arthritis Rheumatol. 2021 Jul;73(7):1347-8. PMID: 33559389. This letter cites the rationale behind dropping “syndrome” in favor of “disease” and demonstrates how our knowledge about Sjögren’s has evolved to a point at which “disease” more accurately describes it.
International Nomenclature Working Group Formed
To ensure worldwide participation, we formed an international working group to engage in scientific deliberation and gain international consensus on nomenclature. The Sjögren’s Foundation’s Vice President of Medical and Scientific Affairs, Kathy Hammitt, MA and its Medical and Scientific Advisory Council Chair, Alan Baer, MD, MACR, Johns Hopkins School of Medicine, were joined by Manuel Ramos-Casals, MD, University of Barcelona, and Sjogren Europe patient representative Coralie Bouillot to bring key opinion leaders and patients in Sjögren’s together.
Delphi Process Methodology to Inform Change
The aim of the nomenclature process was to develop a position paper for the nomenclature and classification of Sjögren’s through international consensus following widespread scientific deliberation. The methodology was a Delphi consensus process, which is a standard and broadly accepted technique for obtaining stakeholder views on a topic and generating consensus among those stakeholders. The process was informed by bibliometric analyses and literature reviews. It was also determined to provide equal weight to votes of patient representatives and clinicians. Read more about the methodology.
The first set of questions around nomenclature was then created and a survey sent to patients and clinicians internationally.
International Symposium on Sjögren’s Syndrome 2022
At the ISSS in Rome in 2022, members of the working group presented the results from the first survey round followed by much discussion and input from both clinicians and patients. There was consensus by all that the initiative should move forward to a second Delphi round.
Second Survey Round
The next set of questions for the second survey round were created based on discussion and input at the ISSS 2022. There were a total of eight questions in the survey. The surveys were sent to Clinicians and Patients around the world (nearly 30 countries participated in the process). There were 81 clinician respondents and 1,454 patient respondents. The results were tallied with equal weighting given to clinicians and patients to determine outcome.
Final consensus on Sjögren’s nomenclature was reached as follows:
- The term Sjögren’s Disease should replace Sjögren’s Syndrome.
- Sjögren’s without “disease” is an acceptable way to refer to the disease, especially once it’s been cited as Sjögren’s Disease.
- The acronym SjD should be used as an abbreviation for Sjögren’s Disease.
- The descriptor “associated” should be used in lieu of "secondary" for Sjögren’s disease occurring in association with a second systemic autoimmune disease for which classification criteria are fulfilled.
- Sjögren's Disease is the preferred terminology in clinical practice, without differentiation as to primary and associated forms. An appreciation of the common association of Sjögren’s with other systemic autoimmune diseases may have value in clinical evaluation of affected patients and in clinical decision-making.
- The differentiation between primary and associated Sjögren’s is recommended for scientific studies to define a homogeneous population.
- The choice of using the possessive or non-possessive form of Sjögren’s and spelling variants (o, oe, ö), should be left to the individual or journal preference.
International Symposium on Sjögren’s Disease 2024
In April 2024, the Sjögren’s community met in Amsterdam at the newly named ISSD conference. The conference opened with the host, Dr. Hendrika Bootsma, announcing that the disease name has been formally changed and that Sjögren’s Disease, replacing Sjögren’s Syndrome, will be used moving forward.
Formal Publication in Peer Reviewed Journal
The formal publication, “2024 International Rome Consensus on the Nomenclature of Sjögren's Disease,” has been submitted for publication and is forthcoming. Of note, consensus initiatives often are named for the location where final consensus was reached – in this case, Rome, Italy – where the 15th International Symposium on Sjögren’s was held in 2022 and nomenclature initiative leadership held in-depth presentations and discussions on nomenclature.
Sjögren’s Disease Name Change History
Read the full history of the name change and how we helped bring the international Sjögren’s medical, research and patient community together.