A group of global experts have endorsed replacing the term “Sjögren’s syndrome” with “Sjögren’s disease” after a consultation with more than 1,400 patients, saying the wording better reflects the nature of the condition.
The 2023 Rome International Consensus on Sjögren Nomenclature published their consensus statement in Nature Reviews Rheumatology [link here] after a comprehensive bibliometric analysis and literature review and a Delphi process including 79 specialists and 1,431 patients from more than 20 countries, including Australia.
The statement makes six recommendations about the naming of Sjögren’s moving forward, including ceasing to use the term “syndrome” to describe the condition and the adoption of “SjD” as an abbreviation for Sjögren’s disease.
The review committee said there were two key reasons behind the name change.
“It is important to recognize that SjD is not a syndrome, namely an aggregate of symptoms and signs that are associated with a morbid process independent of pathogenesis,” they said.
“Instead, SjD is widely accepted as a distinct autoimmune disease, with characteristic autoantibodies, glandular histopathology and a specific pattern of systemic involvement.”
The second argument in favour of a language change came from patients, who were concerned the word ‘syndrome’ could be counterproductive.
It suggested “that SjD is a loose collection of ill-defined or ‘nuisance’ symptoms (overwhelmingly sicca) rather than the actuality, which is that it is a disease with serious morbidity and, for some, increased mortality”, the consensus statement said.
The review also recommended that while Sjögren’s disease was often associated with other systematic autoimmune diseases, it was not necessary to distinguish between “primary” and “secondary” disease unless this was in the context of scientific studies.
“However, this recommendation does not negate the potential value of recognizing the overlap of SjD with other systemic diseases in individual patients and its impact on the attribution of systemic manifestations and treatment choices,” the reviewers said.
The disease was first comprehensively described by Swedish ophthalmologist Henrik Sjögren in 1933, and has been named after him since 1936.
While clinicians and patients both agreed the terminology around the disease was long overdue for change, the Delphi process showed most did not want to abandon the eponym “Sjögren”.
Some experts suggested a new title featuring a histopathological descriptor, like “autoimmune epithelitis”, but this was rejected by patients.
“Patient representatives argued that it would be very difficult for their advocacy organizations to promote awareness and education and obtain increased funding for research with such a technical disease name,” the consensus statement authors said.
The use of “SjD” was also seen as important for conformity and because it was an abbreviation not already commonly used in medical literature.
“Until now, the abbreviation SS (commonly used for Sjögren syndrome) applies to 337 terms85, including some with potentially offensive connotations,” the consensus said.
“Similarly, the abbreviation SD applies to over 209 terms, including the medical terms sudden death, senile dementia and sexual dysfunction. By contrast, SjD is more specific and has only one other medical connotation (sacroiliac joint dysfunction).”
The US advocacy group Sjögren’s Foundation had already abandoned the use of the term.
The group welcomed the publication of the consensus statement, calling it “a pivotal moment for the entire Sjögren’s community”.
“Retiring the term “syndrome” isn’t just a shift in language, it’s a meaningful correction that better reflects the seriousness of the disease and validates the lived experiences of millions around the world.”