If Sjogren’s is a Serious Disease, Why Don’t Doctors Take it Seriously?

Ongoing Care - Part 3

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To get the most out of this post, including finding links to other posts in the Ongoing Care series, be sure to read HOW TO MONITOR SJOGREN'S.  

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INTRODUCTION

For years, I've been trying to understand why doctors pay so much attention to rheumatoid arthritis (RA) and lupus but not Sjogren's. I’ve come to realize that this is less of a problem of individual clinicians, but a system-wide problem, caused by how doctors are educated, how diseases are prioritized, and practical reasons like time and resources. I will address all these factors here and on future blog posts as part of the Ongoing Care series.

Understanding why this is happening will help you better advocate for the care you need. For comprehensive self-advocacy help, see the resources at the end of this post.   

This blog post compares Sjogren’s with RA and lupus. Despite being three closely related systemic autoimmune diseases, RA and lupus receive far more recognition and attention than Sjogren’s disease and here are the main reasons why.

REASONS WHY DOCTORS DON'T TAKE SJOGREN'S SERIOUSLY

1. Doctors are taught that RA and lupus are serious, but that Sjogren’s disease is not.

Doctors are taught that it is crucial to diagnose systemic autoimmune diseases early and aggressively to avoid permanent damage, disability, and death. But many doctors aren’t taught, and don’t know, that Sjogren’s, like RA and lupus, carries a high burden of systemic disease.

In Sjogren’s, “systemic” has a special meaning and refers to non-dryness manifestations, such as arthritis, lung disease, dysautonomia and more. SJOGREN’S IS SYSTEMIC describes why the terms “systemic” and “extraglandular” can be misleading. What doctors know about and think of Sjogren’s varies widely, as I describe on KNOW YOUR RHEUMATOLOGIST. Asking your rheumatologist what they mean when they use the terms can help you understand how they view and care for Sjogren’s and helps you determine how best to advocate for care. 

Unlike RA and lupus, there is no sense of urgency to diagnose or treat Sjogren’s, largely because the “Sjogren’s is a mild dryness disease" myth remains deeply ingrained. This is why Sjogren’s is usually diagnosed in later stages of the disease, leading to a poorer quality of life, disability, and even death. Even after diagnosis, systemic manifestations are routinely overlooked because most doctors have not been trained in all the ways Sjogren’s can impact the body.

When I attended medical school in the 1980s, I was taught that Sjogren’s was a mild dryness (sicca) syndrome that was unimportant to learn about. I know from teaching primary care physicians (PCPs) that they are still being taught that. The “mild sicca disease” myth remains widespread in medicine, despite undeniable evidence that Sjogren’s disease can impact every part of the body and is never “just dryness”.  

2. Doctors are trained to recognize typical presentations of RA and lupus but are not trained to recognize typical presentations of Sjogren’s disease. 

A key mismatch exists between Sjogren’s symptoms, what doctors are trained to identify (dryness), and the systemic signs and symptoms that prompt most patients to seek a diagnosis. Clinicians are almost always more familiar with the presenting signs and symptoms of lupus and RA than they are with those of Sjogren’s disease.

Doctors are taught that the hallmark symptom of Sjogren’s is dryness. But not everyone with Sjogren’s has dryness, or if they have it, they may not notice it, and/or they may not mention it to their doctor. But because dryness is overemphasized in medical education and practice, doctors miss a lot of Sjogren’s diagnoses. Telling a patient who doesn’t have dryness that they can’t have Sjogren’s is like telling a lupus patient they can’t have lupus because they don’t have a classic butterfly rash on their face. 

Early signs and symptoms of Sjogren’s can vary widely, making it all the more important for doctors to be familiar with the common presentations of Sjogren’s other than dryness. One patient might present with painful stiff joints. Another may present extreme fatigue, lightheadedness, and cognitive problems because of undiagnosed dysautonomia. Others may first be diagnosed with lung or kidney disease. If doctors don’t know the impact of Sjogren’s disease on these organs and systems, they will not consider a diagnosis of Sjogren’s.

3. Sjogren’s is often viewed as rare even though it is 3 times as common as lupus and about as common as RA. Doctors are less likely to diagnose a disease if they think it is rare.  Sjogren’s is mistakenly considered only because most people with Sjogren's are undiagnosed. Doctors are less likely to look for diseases they think are rare, and, instead, default to conditions they are familiar with such as fibromyalgia, depression, and anxiety.  

4. The characteristic signs and symptoms of RA and lupus are more visible and measurable than those of Sjogren’s disease, making RA and lupus easier to recognize and diagnose.

RA and lupus usually present with obvious, visible features of their diseases. RA causes swollen joints that can be palpated on exam and seen on X-rays. Lupus often causes a classic butterfly rash as well as other typical skin rashes and mouth ulcers.

In contrast, people with Sjogren’s usually look well and lack obvious, visible signs and symptoms. Arthritis caused by Sjogren’s usually does not cause visible swelling. Neurological, lung and kidney problems caused by Sjogren’s can’t usually be detected by routine physical examination. While Sjogren’s-specific rashes and parotid swelling each impact about 10-20% of patients, doctors may not recognize them as signs of Sjogren’s. It can be difficult for doctors to take Sjogren’s seriously when a patient looks normal, even as they describe feeling desperately ill. There are many more ways that Sjogren’s can be invisible to doctors, which I will discuss in a future post.

5. Unlike RA and lupus patients, Sjogren’s patients often have completely normal blood tests.

RA and lupus patients almost always have one or more positive blood tests that support their diagnosis.

In contrast, people with Sjogren’s have normal or inconsistent blood tests; 30-50% of people with Sjogren’s don’t have SSA, the main blood test used to help diagnose Sjogren’s. Many patients have none of the typical autoantibodies including  ANA, SSA, SSB, or RF. Common markers of inflammation, such as ESR and CRP, are not always elevated, even though Sjogren’s is a systemic inflammatory disease.  ESR is sometimes elevated but CRP is almost always normal.

Please visit MYTHS ABOUT DIAGNOSIS for specific self-advocacy tools, tips, and strategies for advocating for a Sjogren’s diagnosis.

6. Doctors have guidelines for monitoring RA and lupus but have none for Sjogren’s disease. 

Rheumatologists have tools and training to assess disease activity, damage, and progression for RA and lupus but not for Sjogren’s disease.  While researchers use the ESSDAI to measure systemic activity, this tool excludes many important systemic manifestations and is not meant for routine use.

There are no guidelines for monitoring Sjogren’s and there is no standard of care. I started the HOW TO MONITOR SJOGREN’S section and the Ongoing Care blog series to help you talk to your doctors about comprehensive monitoring for Sjogren’s disease.

7.  RA and lupus have FDA-approved drugs. There are no FDA-approved drugs for Sjogren’s. There are multiple FDA-approved drugs for RA and lupus that have been shown by hundreds of well-designed studies to prevent disease progression, permanent disability, and death.

In contrast, Sjogren’s clinical research, including drug trials, lags decades behind diseases like RA and lupus. There are no FDA-approved drugs specific to Sjogren’s disease. However, there are multiple, effective off-label treatments for both systemic and dryness manifestations.  

Some rheumatologists cite the lack of FDA-approved Sjogren’s drugs to rationalize not monitoring or treating Sjogren’s patients. Many are unaware that treatment recommendations are available in the clinical practice guidelines published by the Sjogren’s Foundation.     

See Myth 10 on the MYTHS ABOUT SJOGREN’S page for links to the clinical practice guidelines and ways to advocate for treatments.

CONCLUSION

Sjogren’s is a serious systemic autoimmune disease that, like RA and lupus, requires a timely diagnosis. Every patient should be monitored for a wide range of systemic manifestations so that treatment can begin early, when it is most effective at reducing damage, disability, and even death.

But until doctors are taught to recognize the serious, systemic nature of Sjogren’s, it remains up to each patient to advocate for the medical care they need and deserve.

SELF-ADVOCACY RESOURCES

The book, “How to be a Badass in a Broken Healthcare System”, is a fun, easy read packed with valuable self-advocacy tips from patient advocate Kristina Kelly, who lives with Sjogren’s disease.

“Streamlining Sjogren’s” is a series of videos on how to advocate for care for Sjogren’s disease by Kristina Kelly in consultation with me.  Check out a preview here.

Please use the SJOGREN’S IS MORE THAN “JUST SICCA” handout to advocate for better care of the systemic manifestations of Sjogren’s.