Sarah Schafer, MD
Countering misinformation - MYTH # 1 Sjogren's is mostly dryness, not systemic.
Updated: Feb 1
Sjogren's is a mainly a nuisance dryness (sicca) disease. Systemic involvement is rare.
Sjogren’s is a serious, multisystem disease, never limited to sicca. Nearly every patient who is thoroughly evaluated is found to have at least one systemic (non-sicca) feature as measured by the research tool, the ESSDAI* (38, 92). Systemic features are often overlooked because few patients receive a thorough evaluation. Many systemic features, both common and uncommon, are not included in the ESSDAI (63).
The single most important thing for clinicians to know about Sjogren’s
is that it is a serious, systemic disease. This core reality provides the foundation for comprehensive Sjogren’s care.
THE CONSEQUENCES OF BELIEVING THIS MYTH
Unfortunately, it is common for clinicians to think of Sjogren’s as being an unimportant dryness disease. This misguided view persists largely because of the failure of nearly every medical education institution and rheumatology organization to educate clinicians about Sjogren’s.
Clinicians who view Sjogren’s as a sicca disease are unlikely to monitor for systemic manifestations. Organ involvement such as lung disease and kidney disease may not be recognized until they have advanced to the point of irreversible damage. Better outcomes are associated with appropriate monitoring and timely treatment.
Systemic Sjogren’s manifestations are often downplayed or blamed on other conditions. Psychogenic labels tend to be used when clinicians do not understand that Sjogren’s patients experience numerous scattered features (194).
Sjogren’s neurological manifestations such as small fiber neuropathy and dysautonomia are often attributed to anxiety or “fibromyalgia”. Some patients with these conditions are labeled as having “functional neurological disorder”, the latest term used for conversion disorder. Psychological labels tend to follow the patient for years . They reinforce dismissive attitudes from clinicians.
I was labeled as having conversion disorder when I was later diagnosed with a Sjogren’s neurologic complication. I can’t get it removed from my record.
Not understanding the serious systemic nature of Sjogren’s contributes to delayed diagnosis. Sjogren’s patients with organ involvement or inflammatory arthritis are often misdiagnosed with rheumatoid arthritis or systemic lupus erythematosus and treated for the wrong disease (23). This happens when clinicians are unfamiliar with systemic manifestations, non-sicca presentations (42), and the nuances of Sjogren’s diagnosis,
No one wants to deal with Sjogren’s. No matter the severity of what a doctor has seen, it’s never Sjogren’s that caused it. Scarring in my lung is not related, trigeminal neuralgia is not caused by that, transverse myelitis on MRI isn’t a result of Sjogren’s and on and on.”
Patients want to be seen and believed. Many become reluctant Sjogren’s experts because they cannot count on their doctors to be knowledgeable. Patients may lose trust in the medical system when they cannot get help for this common, serious disease.
I want to be a team with my providers and not have to do all this research. I want to be able to trust them. I’m four years after diagnosis and still not being offered treatment
MYTHBUSTER HANDOUTS FOR CLINICIANS
The clinician handout, Sjogren's Is More Than Just Sicca, attacks this myth head-on. It is not a simple grab-and-go handout. Please read the User Guide to learn strategies for sharing this handout with your clinicians.
See this blog post and article to learn how use the ICD codes as an easy low-key way to educate clinicians.
Sarah Schafer, MD and Sjogren's patient