Sjögren’s name change reflects status as ‘autoimmune disease’
Researchers: Referring to condition as 'syndrome' undermined its serious nature
Renaming Sjögren’s syndrome as Sjögren’s disease is meant to better reflect that it’s a serious autoimmune disease, not just a collection of symptoms, and to offer some clarity across clinical and research settings.
An international group of doctors and patients agreed to the name change in 2023 through a process described in the consensus statement, “2023 International Rome consensus for the nomenclature of Sjögren disease,” published in Nature Reviews Rheumatology. The change and other recommendations described in it were endorsed by the Sjögren’s Foundation and Sjögren Europe.
“Formally updating the name to Sjögren’s disease is more than a terminology shift,” Janet Church, president and CEO of the Sjögren’s Foundation, said in an email to Sjögren’s Disease News. “It is a recognition of the serious, systemic [bodywide] nature of the disease,” which “has been misunderstood or dismissed” for “far too long.” Church said the effort was led for more than a decade by the Sjögren’s Foundation, which was “proud to see the name finally reflect the reality of the disease and the experience of those living with it.”
Sjögren’s is an autoimmune disease that can occur on its own — called primary Sjögren’s — or alongside another autoimmune disease, where it’s referred to as secondary Sjögren’s. Many patients experience dry eyes and a dry mouth, but Sjögren’s can cause symptoms throughout the body.
In recent years, “researchers have highlighted concerns about the inaccuracy of referring to a disease as a syndrome and also the lack of value in distinguishing primary from secondary forms of the disease,” wrote the researchers on behalf of the International Task Force on Nomenclature of Sjögren’s Disease, which was put together after discussions to prepare for the 2022 International Symposium on Sjögren Syndrome.
Formally updating the name to Sjögren’s disease is more than a terminology shift. It is a recognition of the serious, systemic nature of the disease.
In medical terms, a syndrome is a collection of symptoms and signs that tend to occur together and characterize a particular condition, regardless of whether its cause is known. A disease, however, implies a well-defined mechanism. In the case of Sjögren’s, the disease is caused by self-reactive antibodies that mistakenly attack healthy cells.
“Patients have perceived ‘syndrome’ as indicative of a vague collection of symptoms, prompting the Sjögren’s Foundation to abandon the term,” the researchers wrote. For the foundation, using Sjögren’s disease reinforces the idea that it’s a disease for which targeted treatments can be developed.
Reaching consensus on name change
However, when the task force, which was made up of 79 international experts, analyzed the scientific literature, they found that the term Sjögren’s syndrome was used 97.8% of the time among 86,909 medical abstracts published between 1992 and 2022. The possessive form Sjögren’s appeared about three times more often than without it and “there was also a substantial trend over time in the published medical literature away from using ‘secondary Sjögren(’s)’,” the researchers wrote.
To reach a consensus, the task force and 1,431 patients from 23 countries participated in an online Delphi panel, a method that combines anonymous opinions over multiple rounds of surveys. In the first round, most experts (88%) agreed about keeping the condition named after Henrik Sjögren, the ophthalmologist who first described it, and more than half (53%) agreed “syndrome” should be changed to “disease.”
On a 5-point scale, patients gave average scores of 4.35 for maintaining the term “Sjögren” and of 4.23 for renaming it as a disease.
After combining these with the results of a second round, 70% of experts and patients agreed to rename the condition as Sjögren’s disease. They also agreed on “SjD” as an abbreviation for Sjögren’s disease. No effort was made about using the “possessive or non-possessive form of Sjögren in English or about variations in its spelling (o, oe, ö), leaving both matters to “individual or journal preference.”
‘Secondary’ or ‘associated’ Sjögren’s
In the first Delphi round, doctors were also divided on maintaining the term “primary,” but most (73%) rejected “secondary.” A similar proportion (75%) agreed with using “associated” instead. Patients gave average scores of 2.51 for maintaining “primary,” 2.15 for maintaining “secondary,” and 3.08 for changing “secondary” to “associated.”
The task force recommended “associated” be used for Sjögren’s disease when it occurs with another systemic autoimmune disease, called Sjögren disease “the preferred terminology in common parlance and in clinical diagnosis, without differentiation as to primary and associated forms.”
No consensus was reached on the importance of distinguishing primary from associated Sjögren’s in research. Because evidence shows the disease’s features are strongly affected by other autoimmune diseases, the task force recommended that scientific studies state whether associated Sjögren’s patients were excluded or, if not, provide their percentage.
“The scientific rationale for this recommendation is to maintain the comparability of results from new studies with those carried out in the past (practically all of which were carried out in people classified as having ‘primary’ Sjögren),” they wrote. “This consensus marks a significant stride toward a more streamlined and precise understanding of [Sjögren’s disease] within both clinical and research settings.”
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