Name changes for Sjögren’s, axial spondyloarthritis ‘a big deal’ for researchers, patients
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“Ankylosing spondylitis”? “Sjögren’s syndrome”?
These terms may soon take on an antiquated feel not unlike “shell shock” or “consumption,” as both have been targeted for official name changes that better reflect the current understanding of these diseases.
In the case of Sjögren’s, “disease” is replacing “syndrome” to emphasize the serious, systemic nature of this autoimmune disease. Clinicians and patients worldwide made this change to accurately reflect research and patients’ lived experience.
“This is an important change. A ‘syndrome’ is just a collection of symptoms, whereas cumulative research has shown that Sjögren’s is a clearly defined disease,” Janet E. Church, president and CEO of the Sjögren’s Foundation, and a patient with Sjögren’s herself, told Healio.
“The term ‘syndrome’ sounds benign, and it minimizes the significant impact to a patient’s quality of life. For decades, patients have been discounted and have not received the focused treatment they need. This name change sets the stage for health care providers to accurately diagnose and treat patients, understanding that Sjögren’s is a serious disease.”
Meanwhile, “axial spondyloarthritis” — or its five-letter abbreviation “axSpA” — has come to encompass the whole spectrum of disease formerly divided into non-radiographic vs. radiographic axial SpA, also known as ankylosing spondylitis.
“The most important reason for the change was to recognize that ankylosing spondylitis doesn’t just start with radiographic evidence of sacroiliac joint damage,” the Axial Spondyloarthritis International Federation (ASIF) told Healio in a statement. “While it cannot be detected by X-ray, it can be detected by MRI.”
From ‘syndrome’ to ‘disease’
The goal of shifting from “Sjögren’s syndrome” to “Sjögren’s disease” is to ensure that people know Sjögren’s is serious, systemic and that patients need treatment and long-term multidisciplinary care, according to Church.
“For those in the health care profession who have even heard of Sjögren’s before, they have likely been incorrectly taught that it is just a simple dry-eye, dry-mouth disease, and that’s not the case,” she said. “Sjögren’s patients experience a wide range of symptoms and disease severity that can drastically impact and alter their lives.”
Dry eyes and mouth, or sicca, are the most widely known Sjögren’s symptoms, but nearly all patients also face systemic manifestations, such as joint pain, debilitating fatigue, neuropathies, lymphomas and gastrointestinal and lung conditions. Many people also believe Sjögren’s is rare, despite it being among the most common autoimmune diseases in the United States.
Changing “syndrome” to “disease” conveys the seriousness of the condition and indicates it has been established as a distinct pathology that cannot be waved away, Church said. Similar motivations are also driving a shift from “secondary Sjögren’s” to the more neutral “associated Sjögren’s” when patients demonstrate other rheumatic diseases.
The Sjögren’s Foundation has been working for nearly 9 years to solidify changes to the Sjögren’s nomenclature. The organization removed the word “syndrome” from its own name in 2019 and, in 2021, called for wider adoption in a letter to the editor published in Arthritis & Rheumatology, written by Alan Baer, MD, MACR, chair of the Sjögren’s Foundation medical and scientific advisory council, and Kathy Hammitt, MA, vice president of medical and scientific affairs at the Sjögren’s Foundation.
The Sjögren’s Foundation soon convened an international working group to hammer out consensus on new terminology, led by Baer; Hammitt; patient representative Coralie Bouillot; and Manuel Ramos-Casals, MD, of the University of Barcelona, who had also been working independently for nomenclature changes. The task force’s work involved bibliometric analyses, a systematic literature review and multiple rounds of the Delphi consensus process, which saw 1,454 patients and 81 providers surveyed around the world.
Along the way, there were suggestions that the name — a reference to Henrik Sjögren, the ophthalmologist who first described the disease — be scrapped in its entirety, according to Baer.
“Some Sjögren’s clinicians or investigators argued for the abandonment of the eponym and the use of a descriptive name, such as autoimmune epithelitis or autoimmune exocrinopathy,” he said. “However, the patients were opposed to a descriptive name, since the term ‘Sjögren’s’ was already well-established and conveyed quite simply a complex disease construct. A small minority of clinicians or investigators argued for maintaining the term ‘syndrome.’”
The process also saw disagreement about whether “secondary Sjögren’s” should be replaced with “associated Sjögren’s” in cases when other rheumatic diseases are present. Patients felt that referring to coexistent Sjögren’s as “secondary” diminished its importance to their clinical picture, when “often it is the most debilitating disease” they face, Church said.
According to the Sjögren’s Foundation website, “all patients wanted the term [secondary] to be abolished,” while some clinicians had reservations about smudging what they saw as a useful distinction. Baer was among the latter group.
“There are clinicians who prefer to be ‘lumpers,’ rather than ‘splitters,’” he said. “There was disagreement among the clinicians and investigators as to whether this distinction was important in clinical practice, or simply in the research area. I felt that it was important for both, but not everyone agreed with me.”
Some finer details also were settled during the process. The preferred abbreviation is SjD; the word “Sjögren’s” alone is acceptable; and the choice of spelling variants (ie, Sjögren’s vs. Sjogren’s vs. Sjoegren’s) is up to individual preference.
A formal paper announcing the final consensus, along with a description of the process behind it, is now under peer review, according to the Sjögren’s Foundation. Because much of the in-depth discussion occurred in 2022 during the 15th International Symposium on Sjögren’s in Rome, the paper is titled, “2024 International Rome Consensus on the Nomenclature of Sjögren's Disease.”
According to Church, some electronic health record systems are already switching over to the use of “Sjögren’s disease.” Experts from the international working group are also expected to pursue this change elsewhere, including in the International Classification of Diseases. NIH agencies have also begun changing to “Sjögren’s disease.”
“For a full and complete name change, it keeps rolling down the hill, with the ball of yarn getting bigger until the impact is made,” Church said. “The very first step of that is, really, the clinician publication.”
Church added that she hopes that the name change — along with ongoing clinical trials, including phase 3 studies by Amgen, Bristol Myers Squibb and Novartis — will prompt doctors to “take a fresh look” at Sjögren’s.
“The opportunity to communicate this change will hopefully awaken all health care providers to say, ‘Oh, what’s this? Why did this happen? Let me learn about the current information about Sjögren’s so I can better diagnose and treat patients,’” she said.
Consolidating axSpA
The name of the condition known for decades as ankylosing spondylitis — and, over the years, subdivided as radiographic or non-radiographic axSpA — has been streamlined.
The overarching name of the disease is now “axSpA.” From there, distinctions such as whether disease activity is visible on X-ray (ie, radiographic vs. non-radiographic) “may be relevant for research purposes.”
All this according to an editorial heralding the change, published in November 2023 by the Assessment of Spondyloarthritis International Society (ASAS) in Annals of the Rheumatic Diseases.
“What a provider should know is that axSpA includes the non-radiographic stage, and so, for diagnostic purposes, there is no need to diagnose people, as has been the case, with nr-axSpA,” ASIF, a separate axSpA advocacy group, said in a statement.
The group additionally argued that the new term may also make the disease easier for outsiders to understand.
“We believe that having ‘arthritis’ as part of the name can make it easier for patients to understand and to explain to others,” the ASIF statement continued. “Arthritis is a widely recognized disease, and it can help provide even a very basic understanding of what the disease may be.”
Likewise, the word “ankylosing” could have given “the wrong impression to patients that the spine will become fused, while this is only in a minority of the patients,” Désirée van der Heijde, MD, PhD, co-author of the ASAS editorial and professor of rheumatology at Leiden University Medical Center, in the Netherlands, told Healio.
The story of how ankylosing spondylitis became axSpA actually stretches back to the 1980s, when the adoption of clinical MRI yielded new insights into the disease’s course.
“It was realized that sacroiliitis, a hallmark feature of the disease, could be detected long before any damage from it could be seen on X-ray,” the ASIF said in its statement. “In fact, sacroiliitis could be detected on MRI about 18 to 24 months after disease onset, whereas the damage from it takes 7 to 8 years or so after disease onset to be seen on X-ray.”
In light of this, the term “non-radiographic axial spondyloarthritis” (nr-axSpA) was coined to describe the period when disease activity is not visible on X-ray. However, debates continued as to whether it was truly the same disease as ankylosing spondylitis, ASIF stated.
However, by 2019, the annual ASAS meeting saw majorities in favor of consolidating the nomenclature, according to the group’s editorial. There was a near-unanimous 99% vote that axSpA should be the overall name of the disease; an 82% majority declaring “ankylosing spondylitis” and “radiographic axSpA” to be interchangeable terms; and 55% agreement that the latter is the preferred term.
The votes were informed by the findings of two ASAS research initiatives. The first was a systematic literature review and meta-analysis finding broad similarities between radiographic and non-radiographic axSpA and supporting their classification as two points on the spectrum of a single disease. The other, a study of nearly 4,000 patients, found substantial overlap between diagnostic criteria for ankylosing spondylitis and radiographic axSpA, supporting their interchangeability.
The process culminated in ASAS’ 2023 editorial urging readers to bid “goodbye” to ankylosing spondylitis and “hello” to axial spondyloarthritis.
So far, trickle-down effects of the name change include the renaming of a major disease activity index, the ASDAS, which now stands for the Axial Spondyloarthritis Disease Activity Score. The condition is also now listed in the ICD-11 as axial spondyloarthritis, with ankylosing spondylitis included as a matching term.
The nomenclature change will also widen the prospects for clinical trials, according to Philip J. Mease, MD, director of rheumatology research at Swedish Medical Center/Providence-St. Joseph Health, in Seattle, who recently explained his position in a presentation at the 2024 Congress of Clinical Rheumatology West.
Dubbing non-radiographic axSpA a “somewhat silly and arbitrary concept,” Mease argued that bringing it under a single umbrella with radiographic axSpA means researchers will no longer have to subdivide between the two patient groups.
“We can just do one big study,” he said.
Lumping non-radiographic and radiographic disease together also leads to a different impression of axSpA’s prevalence, Mease added.
“We’ve got pretty good evidence that, in the United States, ankylosing spondylitis constitutes 0.5% of the population, and it’s two-to-one male vs. female,” he said. “If we broaden the concept to the full axial SpA categorization, that number goes from 0.5% up to 1.4% of the population, and it’s now become equigender and is no longer male predominant.”
All of that said, the changeover has not in all matters been smooth. For example, the ASAS editorial acknowledged ongoing confusion stemming from “continuous use of both terms.”
Persistent references to ankylosing spondylitis “will be hard to change,” Robert B.M. Landewé, MD, PhD, a co-author of the editorial and associate professor of rheumatology at the University of Amsterdam, told Healio.
“It will probably take a generation and a change of textbooks to really adopt the new terminology,” he said, adding he expects it to be “a while before axSpA will sink in.”
According to Landewé, the old terminology is “only wholly out of date when people do not use it anymore.”
“I am afraid that the term AS will sustain for a while, since historically drugs have been approved by the FDA and European Medicines Agency for the disease ‘AS,’” he said. “Later on, there were additional approvals for ‘non-radiographic axSpA,’ which in my mind always sounds a bit redundant. This will be hard to change.”
Rheumatologists have largely adopted the new nomenclature over the last decade, with conference posters and presentations shifting over to axSpA, but “uptake is slower” among non-specialists, the ASIF stated.
“The name ‘ankylosing spondylitis’ has been used for a century and is so entrenched that it will continue to be used by both providers and patients for some time to come,” the organization added.
References:
Language Matters! The International Sjögren’s Community Changes Sjögren’s Syndrome to Sjögren’s Disease! https://sjogrens.org/blog/2024/language-matters-the-international-sjogrens-community-changes-sjogrens-syndrome-to. Published Sept. 24, 2024. Accessed Friday, Oct. 18.
van der Heijde D, et al. Ann Rheum Dis. 2023;doi:10.1136/ard-2023-225185.
For more information:
Janet E. Church can be reached at jchurch@sjogrens.org.
Alan Baer, MD, MACR, can be reached at alanbaer@jhmi.edu.
Désirée van der Heijde, MD, PhD, can be reached at mail@dvanderheijde.nl.
Robert B.M. Landewé, MD, PhD, can be reached at landewe@rlandewe.nl.
The Axial Spondyloarthritis International Federation can be reached at office@asif.info.
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