Sarah Schafer, MD
Sjogren's is Sjogren's; it is not secondary to any other disease you may have.
Have you been diagnosed with “secondary Sjogren’s”?
“Secondary Sjogren’s” has long been used to describe Sjogren’s that occurs together with another connective tissue disease (CTD), such as rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE). Despite widespread use, the term “secondary” is outdated, inaccurate and misleading. The correct term is “associated Sjogren’s.”
If your rheumatologist calls your Sjogren’s “secondary”, it is a good idea to ask if they manage your disease the same way they would manage Sjogren’s alone (previously called “primary”). If they downplay Sjogren’s because it is “secondary”, you might not get the care you need.
Call it “associated Sjogren’s”.
Sjogren’s experts are encouraging rheumatologists to use the term “associated Sjogren’s” instead of “secondary Sjogren’s”. While it may seem trivial to insist upon proper terminology, rheumatologists may use the term “secondary” to mean that Sjogren’s is of secondary importance to your other “more serious” CTD(s). This reinforces the incorrect idea that Sjogren’s is not a serious multisystem disease.
For many years, “secondary Sjogren’s” was assumed to be a milder form of Sjogren’s. This is not the case. We now know that the disease characteristics are the same as Sjogren’s alone when you account for the overlapping features from the associated CTD(s) (122, 123, 153) Patients with associated Sjogren’s, just like Sjogren’s alone, should be offered comprehensive monitoring and management.
Sjogren’s deserves specific attention, regardless of how many CTDs the patient has.
If you have both RA and SLE, no one calls it “secondary SLE” or “secondary RA”, regardless of which disease was diagnosed first. The same should apply to Sjogren’s. Sjogren’s is Sjogren’s. If you have Sjogren’s with SLE or RA, you have both diseases. If you have all three, you have all three. You need to address each one and not downplay one or the other. (42, 153)
Sjogren’s does not arise from another CTD.
The term “secondary” probably started when Sjogren’s was thought to arise from a so-called “primary disease”. Sjogren’s, SLE, RA, systemic sclerosis, and a few other CTDs do share some (but not all) genetic markers. Similar biologic pathways and triggers may lead to disease (123). It is not surprising that these diseases tend to occur together. Having similar etiologies does not mean that one disease causes the other.
Sjogren’s does not turn into SLE (lupus).
The incorrect idea that Sjogren’s becomes SLE is a holdover from decades prior, when Sjogren’s was sometimes thought to be a milder form of SLE. This historical artifact needs to be discarded. A Sjogren’s diagnosis may precede or follow another CTD diagnosis. A Sjogren’s patient who develops SLE simply has two major diseases and vice versa.
What can you do if you think that your Sjogren’s care is being neglected or overshadowed by another CTD?
Ask your rheumatologist if they think Sjogren’s is less important than your other disease(s).
Share this printable Sjogren’s Quarterly article, “Why Language Matters”, with your clinician as a way to discuss your concerns. You might say, “I saw that this recent Sjogren’s Foundation article recommends not calling it ‘secondary Sjogren’s’. What do you think about that?” This open-ended question may reveal a lot about their view of Sjogren’s. It is less likely to put them on the defense than asking them why they are calling it “secondary”.
If your rheumatologist seems interested and receptive, invite them to subscribe to the Sjogren’s Quarterly newsletter. It is available free of charge to clinicians at www.sjogrens.org.
Why say anything at all? Does "secondary Sjogren's" really matter? YES!
“Secondary Sjogren’s” is not just one bad term that exists in a vacuum. It illustrates the bigger problem of Sjogren’s being downplayed and dismissed, rather than being treated as the common, serious, systemic disease that it is. I call this Sjogren’s neglect.
Advocate for change with the words you use.
As patients we can have an impact, especially when many of us speak up. Each action we take, big or small, to shift how the rheumatology community thinks about (or ignores) Sjogren’s makes a difference.
We can remind our own doctors that words matter. We can diplomatically point them to resources that reflect the current understanding of Sjogren’s. This in turn can lead to better care.