Sarah Schafer, MD
Why doesn't my doctor understand Sjogren's?
Updated: Sep 20, 2022
Sjogren’s it is a serious systemic disease, impacting about 1% of the U.S. population (79). Every clinician should know the basic facts about this common disease. Yet this is often not the case. While sicca (dryness) symptoms often appear early, systemic (non-sicca) features are what typically drive patients to seek a diagnosis. Primary care providers (PCPs) and specialists such as neurologists, pulmonologists, and others are usually the first to be encounter systemic Sjogren’s features.
Sjogren’s has a paradigm problem.
A paradigm refers to a typical way of looking at something. Sjogren’s is widely, and incorrectly, viewed as being a rare, sicca-limited disease. Clinicians who believe the “nuisance sicca disease” myth are being led astray by outdated, inaccurate assumptions about the fundamental nature of the disease. The negative impact of this outdated view of Sjogren's cannot be overstated. Extensive delays to diagnosis are the norm. Symptoms are often downplayed, dismissed, or psychologized. Clinicians routinely underestimate the burden of the disease and fail to address the wide variety of systemic Sjogren’s manifestations.
Over the past decade, researchers have demonstrated that Sjogren’s is undeniably a systemic disease. Ramos-Casals et al evaluated nearly 1000 Sjogren’s patients for systemic features, using the research tool, the ESSDAI (EULAR Sjogren’s Syndrome Disease Activity Index). They demonstrated that 70-80% of Sjogren’s patients have at least one systemic feature at the time of diagnosis and 90 % have at least one after six years (38).
Even the high numbers found in this study underestimate the extent of systemic involvement. The ESSDAI is an excellent research tool but it does not measure every systemic Sjogren’s manifestation (63, 112). ESSDAI evaluations do not routinely assess patients for common neurological manifestations such as small fiber neuropathy and autonomic disorders. Patients are not routinely screened for lung disease, which is now recommended by the Clinical Practice Guidelines for Sjogren’s Pulmonary (lung) Disease. Sjogren’s lung disease is far more common than previously thought. It can be detected in a sizable number of asymptomatic patients (89, 158).
Patients with systemic Sjogren’s manifestations at the time of presentation may not have obvious sicca symptoms (3, 8). Sjogren’s patients with organ involvement or inflammatory joint pain are frequently misdiagnosed as having systemic lupus erythematosus (SLE) or rheumatoid arthritis (RA) because clinicians do not realize that these also occur in Sjogren’s (23).
“Sjogren’s is a systemic autoimmune disease that affects the entire body” ~ The Sjogren’s Foundation
Why are so many clinicians entrenched in the “nuisance sicca disease” model of Sjogren’s?
The main reason: Medical education about Sjogren’s is largely non-existent. The Sjogren’s Foundation does what they can, but they cannot meet this challenge alone. Because there are so few Sjogren's updates, rheumatologists who do stay up to date usually do so on their own initiative. Widespread Sjogren’s medical education is essential for improving Sjogren’s care. Many rheumatologists remain unaware that Sjogren’s Clinical Practice Guidelines exist. There are no consistent standards of practice.
For every Sjogren’s clinician education program, there are hundreds for RA and SLE, two diseases closely related to Sjogren's. This disparity gives clinicians the message that Sjogren’s is unimportant. PCPs are almost always under the impression that Sjogren's is an uncommon sicca disease. It is simply not on their radar. I have taught more than 200 PCPs about Sjogren's over the past decade. I start my sessions by asking them how they view Sjogren's. Almost every PCP was unfamiliar with even typical presentations. Only a handful could name even one systemic manifestation (the few that did named fatigue).
Some clinicians put Sjogren’s in the “too hard basket”. Sjogren’s is complex and time consuming to manage. Clinical manifestations are extremely variable and often require care from multiple specialists. There is no “one size fits all” treatment strategy. No single medication helps every patient or every feature. In my experience, most clinicians want to provide the best care possible. The deficits in Sjogren’s care reflect a system-wide neglect of the disease. This is a much bigger problem than any individual clinician’s practice. For individual clinicians, knowledge, patience, creativity, and humility are required to diagnose and treat this challenging disease.
Institutional barriers to comprehensive Sjogren’s care. Prior to October 2021, the ICD- 10 billing code for Sjogren’s was “sicca syndrome”. This misleading term reinforced the incorrect view of Sjogren’s as a sicca disease. Thanks to the Sjogren’s Foundation, the ICD- 10 codes now reflect the systemic nature of the disease.
Rheumatologists and PCPs receive little training or support for Sjogren’s care. It can be tricky to offer educational materials that go against their current thinking. This can backfire if patients do not choose materials wisely and use a collaborative approach. Please see the Clinician Handouts page for how to share Sjogren’s information with clinicians.
Handouts for countering the “mild sicca disease” myth are included in the blog post, Countering misinformation, MYTH #1. More handouts are in the pipeline.