Have you been diagnosed with “secondary Sjogren’s”?
“Secondary Sjogren’s” has long been used to describe Sjogren’s that occurs together with another autoimmune rheumatic disease (AIRD) such as rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE). Despite widespread use, the term “secondary” is outdated, inaccurate , and misleading. Sjogren's is a unique disease whether or not it occurs with another AIRD or immune-mediated disease.
Rheumatologists often refer to AIRDs as connective tissue diseases (CTDs), although this term outdated and non-specific.
It is important to understand how your rheumatologist's view of "secondary" impacts your care.
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.
While it may seem trivial to worry about proper terminology, rheumatologists often use “secondary” to mean that Sjogren’s is of secondary importance to other diseases they consider more serious, such as RA or SLE. Sjogren' is serious and requires specific monitoring and management. It is never just dryness.
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 as for Sjogren’s alone when you account for the overlapping features from the associated AIRD (122, 123, 153). Sjogren's patients should be offered comprehensive monitoring and management for Sjogren's even if they have another AIRD. For example, Sjogren's is much more likely to cause dysautonomia, small fiber neuropathy, and lymphoma than the other AIRDs.
Sjogren’s deserves specific attention, regardless of how many AIRDs 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. It is simply referred to as having RA and SLE. The same should apply to Sjogren’s. You need to consider each disease for clinical management (42, 153). Sjogren's care should never be limited to dryness (sicca) management. Sjogren's is always systemic and never limited to dryness.
Sjogren’s does not arise from another AIRD.
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 AIRDs 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 AIRD diagnosis. A Sjogren’s patient who develops SLE simply has two major diseases and vice versa.
TIP: What can you do if you think that your Sjogren’s care is being neglected or overshadowed by another AIRD?
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.
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.
If we all choose to act, we will make a difference!
