Countering misinformation - MYTH # 1 Sjogren's is mostly dryness, not systemic.
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 upcoming handout, Sjogren’s is Always Systemic addresses this myth head-on. It is not published yet because detailed instructions are important for effective use. This blog post provides important background for the new handout.
Other materials that you can share with clinicians: Please be sure to read the Clinician Handouts page for tips. Remember that Sjogren's Advocate pages and blog posts are not meant to be used as clinician handouts.
1. ICD 10 codes article: the new billing codes document many systemic manifestations of Sjogren’s. See this blog post, to learn how use the ICD codes as an easy low-key way to educate clinicians.
Read this article to the best of your ability. Click on the "Full text link", then use that page to print the PDF version. Highlight the following sentences in the abstract and point them out to your clinician. Results: “The cohort consisted of 921 patients. After a mean follow-up of 75 months, 77 (8%) patients still had an ESSDAI* score of zero at the last visit.” My comment: this means 92% had at least one ESSDAI feature at 75 months (about 6 years). Conclusion: “Primary SS is undeniably a systemic disease, with the joints, lungs, skin and peripheral nerves being the most frequently involved organs.”
*See this page for a description of the ESSDAI, a standardized research tool that measures systemic activity. Because it does not include many common systemic features or stable systemic manifestations, the ESSDAI score underestimates systemic involvement.
Have you tried sharing Sjogren’s educational information with your clinicians? Please send comments using the email on the contact page found by scrolling down to the very bottom of any Sjogren’s Advocate page.
Sarah Schafer, MD and Sjogren's patient