Words matter: problematic Sjogren's disease terms
Updated: Sep 29, 2020
Some medical terms used to describe Sjogren’s disease are ambiguous. Some are downright misleading. This can lead to miscommunication during appointments or even confusion within the medical community.
Language frames the way we think about a topic. Words matter. They can reinforce the dismissive attitudes toward the disease and to the patient. It may be important to clarify confusing terms with your clinician to make sure you are on the same page. Some of the biggest offending terms are listed below.
Lay people often have trouble looking up Sjogren’s information on the internet because they cannot remember the name of the disease or spell it. Even physicians can be intimidated by the umlaut in the name (which I intentionally leave out.) We are probably stuck with the “Sjogren’s” part of the name. When I teach, I often mention that medical literature searches work fine without umlauts or apostrophes.
“Syndrome” is the most problematic aspect of this unfortunate disease label. "Syndromes" are often thought of as serious, or perhaps not even real. Yet Sjogren’s is a well-characterized autoimmune disease, not a mysterious bucket of symptoms. Sjogren’s Disease is a far more appropriate name, and this term is slowly replacing “Sjogren’s Syndrome” in the literature.
In this website, I use the shorthand “Sjogren’s” because it flows more smoothly in the writing. However, when I talk with clinicians, I often use the term “Sjogren’s Disease,” to reinforce the serious, systemic nature of the disease. The Sjogren’s Syndrome Foundation is soon to be called the Sjogren’s Foundation for this very reason.
Sicca syndrome and keratoconjunctivitis sicca (KCS)
There are many people who have sicca but do not have Sjogren’s. It is fine to use these terms in those situations. It is also fine to say “Sjogren’s with predominant KCS.” However, once Sjogren’s is diagnosed, the disease itself should not be referred to as KCS or Sicca syndrome. These terms perpetuate the misperception of Sjogren’s as a nuisance dryness disease. KCS and sicca symptoms are just one aspect of a very complex systemic disease.
Primary and secondary Sjogren’s
These terms are problematic on so many fronts. “Secondary Sjogren’s” means different things to different rheumatologists and researchers. When this term is used in Sjogren's research papers, you have to determine the authors’ definition of Secondary. More importantly, “Secondary” reinforces the incorrect notion that Sjogren’s is of secondary importance to diseases that often co-occur, such as rheumatoid arthritis and systemic lupus erythematosus. Sjogren’s is Sjogren’s, whether it occurs alone or with another major rheumatologic disease. The new correct terminology is “Sjogren’s with or without associated disease.” Then simply name the associated disease(s).
This unfortunate term is used in the literature to refer to the features that patients find to be the most disabling aspects of Sjogren's: fatigue, pain, stiffness and brain fog. Physical and cognitive fatigue are often the primary reasons for unemployment and low quality of life (27). A better term is "General systemic features."
"Benign manifestations," should never be used when describing Sjogren’s. We need to be clear with our clinicians that these serious Sjogren’s features are anything but benign.
Extraglandular Manifestations (EGM)
There is a lack of widespread agreement on the exact meaning of this term. See Always Systemic for a detailed explanation about this confusing term, including important systemic features missing from the classic EGM list.
In Sjogren’s, the term “seropositive” technically refers to patients who are SS-A positive. However, many rheumatologists use this loosely. Some will call ANA positive or SS-B positive Sjogren’s patients “seropositive” even if the test for SS-A is negative. I was recently told that internists often consider the term “seropositive” to be synonymous with positive rheumatoid factor (RF), regardless of the disease. Go figure!
This term has classically referred to SS-A and SS-B. However, these antibodies are not actually specific to Sjogren’s. SS-A and/or SS-B are fairly common in systemic lupus and systemic sclerosis (6, 46, 59) and may occasionally be seen in healthy people without evidence of autoimmune disease. The actual role of these antibodies in the disease process is unclear, especially since so many Sjogren’s patients (30-40 %) do not carry them.
Classification Criteria are NOT diagnostic criteria
The term, Classification Criteria, is fine when clinicians understand what it means and how it should be used. However, many clinicians incorrectly think of Classification Criteria as diagnostic criteria. Currently, there are no diagnostic criteria for Sjogren's disease.
Classification Criteria were created in order to standardize research populations. A significant number of Sjogren's patients do not meet these necessarily strict Classification Criteria. Sjogren’s is a clinical diagnosis. Testing supports but does not rule out the disease. Clinicians unfamiliar with the nuances of diagnosis often refuse to see or treat Sjogren’s patients who do not fulfill these narrow research criteria. This leaves many with Sjogren’s undiagnosed and untreated.
Why do these terms matter?
It is important to check in with your clinician when they use these terms. For example: If they use the term, seropositive, ask “Do you mean SS-A positive when you say seropositive?” You might be surprised by the answer.
If they use the term “diagnostic criteria” you can say: “Do you mean the 2016 Classification Criteria?” They may not even be familiar with these latest criteria, especially if they insist that positive blood tests are necessary for diagnosis.