Sjögren's disease is preferable in both clinical and research settings.

The preferred nomenclature for the condition formerly known as "Sjögren's syndrome" has been changed to Sjögren's disease, according to an international consensus statement published in Nature Reviews Rheumatology.

The authors of the statement, including Alan Baer, MD, professor of medicine and founder of the Jerome L. Greene Sjögren's Disease Center at Johns Hopkins University, described the use of the term syndrome as "unsatisfactory."

"The term 'syndrome' denotes an aggregate of symptoms and signs that are associated with a morbid process, independent of pathogenesis," Baer told Healio. "'Viral syndrome' and 'chronic pain syndrome' are good examples.

"In contrast, Sjögren's is now widely accepted as a distinct autoimmune disease, with characteristic autoantibodies, glandular histopathology and a specific pattern of systemic involvement," he added. "Second, those affected overwhelmingly felt that the term 'syndrome' implied that their disease was nothing more than a loose collection of ill-defined or nuisance symptoms -- overwhelmingly sicca -- rather than a disease with serious morbidity and, for some, increased mortality."

After an exhaustive literature review, the authors convened an international group of more than 90 health care professionals and 1,400 patients to reach a consensus on a name.

In addition to the shift from disease to syndrome, they also recommend the use of "SjD" as the preferred abbreviation. Moreover, they eliminated the use of terms describing "primary" and "secondary" Sjögren's, and offered guidance for both clinicians and researchers in the field.

Healio sat down with Baer to discuss the rationale and timing of this statement, the importance of nomenclature in both clinical and research settings, and the specific components of their recommendations.

Healio: Why is such nomenclature important?

Baer: Symptoms of ocular and oral dryness are common in the population and the subset of people with these symptoms occurring in tandem are often labeled as having sicca syndrome. It is important to distinguish those who have Sjögren's disease from the larger pool of people with sicca syndrome, since there are distinct prognostic implications and potentials for immunomodulatory therapy.

Healio: Among members of the Sjogren's disease community -- including physicians and patients -- this was a long time coming, but why now?

Baer: This change was spurred in part by the decision to change the name of the Sjögren's Syndrome Foundation to the Sjögren's Foundation in 2019, for the reasons I just mentioned. This was followed by a letter to the editor entitled "Sjögren's disease, not syndrome," published in Arthritis & Rheumatology in 2022, in which Kathy Hammitt of the Sjögren's Foundation and I called for the name change.

However, it was clear to us that international consensus was required for the name change to be accepted by medical journals and organizations, including the American College of Rheumatology. We were thus delighted to join forces with Manuel Ramos-Casals, MD, PhD, of the University of Barcelona, Spain, and use the 2022 International Sjögren's Conference in Rome as a forum to achieve consensus on the nomenclature and classification of the Sjögren, similar to the conference held in Chapel Hill, North Carolina, to reach consensus on the classification of vasculitis.

Our objective was to launch an expert-based discussion about certain terms -- syndrome vs. disease, primary and secondary disease vs. associated -- and classification issues, such as association vs. overlap, relationships with other systemic and organ-specific autoimmune diseases, and with non-autoimmune processes, and to write an international consensus document.

Healio: Was there anything remarkable or noteworthy about the literature review as you began to put this document together?

Baer: When Sjögren's disease occurs in association with another systemic autoimmune disease, there is substantial alteration of both the epidemiological profile and the phenotype of Sjögren's, compared with the characteristics observed in the absence of another coexisting disease.

Healio: Who was involved with this effort, and how were those individuals chosen?

Baer: Our Delphi consensus process was unique in that it involved both medical professionals with expertise in Sjögren's as well as patient representatives, with both groups receiving equal weight in the voting process.

The experts included 94 medical professionals from multiple specialties, including rheumatology, internal medicine, oral medicine, ophthalmology and pediatrics, across 28 countries. They were selected based on their established record of clinical research in Sjögren's.

The opinions of 1,431 patients with Sjögren's disease from 23 countries were canvassed using email lists of members of the U.S. Sjögren's Foundation, Sjögren Europe and Sjögren's Society of Canada.

Healio: What does this name change mean for patients with Sjögren's disease?

Baer: The name change serves to underscore the serious and systemic nature of the disease.

The term "secondary Sjögren's" is now abandoned, since it connoted that Sjögren's was "less than" the other diseases. Notably, the term "secondary" is never applied to diseases such as systemic lupus or rheumatoid arthritis when they are associated with Sjögren's.

Healio: What does this name change mean for researchers investigating Sjögren's disease?

Baer: The distinction between primary and secondary forms of Sjögren's has had substantial consequences for research, as most published scientific studies in Sjögren's focus on the primary form, excluding patients with an associated systemic autoimmune disease.

Despite the relatively common overlap of Sjögren's with other systemic autoimmune diseases, there is a paucity of research on the clinical features and optimal clinical management of these overlap patients. We hope to see more research on this important subset of patients with Sjögren's.

Our systematic literature review demonstrated that the coexistence of another systemic autoimmune disease heavily influences the phenotype of Sjögren's. Thus, the inclusion of patients in a study of Sjögren's and an overlap disease, such as lupus or RA, is encouraged but may require specific sensitivity analyses post-hoc.

Healio: What should rheumatologists and other health care providers know about this name change, and why is it important to their work?

Baer: With the development of disease-modifying therapies for Sjögren's, it is important that rheumatologists and other health care providers recognize that Sjögren's is a systemic autoimmune disease with distinct clinical and pathologic features. Accurate diagnosis of Sjögren's disease will ensure that these drugs, when available in the near future, will be prescribed for those whose sicca symptoms and systemic manifestations have an autoimmune basis.

An appreciation of the common association of Sjögren disease with other systemic autoimmune diseases also has value in the clinical evaluation of affected patients and in clinical decision-making.

Healio: How did you arrive at Sjögren's disease, as opposed to some other nomenclature?

Baer: Through a Delphi process, consensus was reached on a few counts. One was to maintain the eponym Sjögren as opposed to switching to a descriptive disease name, such as autoimmune exocrinopathy or autoimmune epithelitis. The second was to change the term from syndrome to disease.

The third was to use the term "Sjögren disease" as opposed to alternatives such as the novel acronym 'S.J.Ö.G.R.E.N.' -- salivary gland swelling, joint pain/swelling, ocular/oral dryness, general symptoms, renal/respiratory manifestations, exocrinopathy, non-Hodgkin lymphoma -- or Sjögren autoimmune epithelitis and Sjögren autoimmune exocrinopathy.