Words matter: problematic Sjogren's disease terms
Updated: Oct 18, 2019
Some medical terms used to describe Sjogren’s disease are ambiguous. A few 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 offenders 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. 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. People diagnosed with “syndromes” are often written off as not having something serious, or not having a real disease. Yet Sjogren’s is a well characterized disease, not a mysterious bucket of symptoms. Sjogren’s Disease is a far more appropriate name, and 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,” and ask them to use that term. This helps 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 the tip of the iceberg for the underlying 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 Sjogren’s Foundation has an excellent analysis of why these terms should not be used. 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 the disease: 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)
In Sjogren’s, the term “seropositive” technically refers to patients who are SS-A positive. However, many rheumatologists 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 many internists 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, which do not actually exist. Classification Criteria were created in order to standardize research populations. A significant subset of patents with Sjogren’s disease 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 fulfull these narrow research criteria. This leaves many with Sjogren’s undiagnosed and untreated. See the excellent explanation about this from the Sjogren’s Foundation.
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 at 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.