Thread by @SarahSchaferMD

#Sjogren’s patients often have difficulty finding the care they need, especially when it comes to systemic manifestations. Many are told that they have the “glandular version”, despite undeniable evidence that Sjogren’s is a serious, systemic disease.
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There is no “glandular version” of Sjogren’s. You can find systemic features in almost every patient- if you look for them.
“The evaluation of the systemic manifestations of SS are not properly incorporated into clinical practice”.
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pubmed.ncbi.nlm.nih.gov/35650656/

When Sjogren’s is viewed as a nuisance sicca (dryness) disease, patients are not monitored for systemic manifestations. There are no guidelines for the routine monitoring of Sjogren’s. Until we have clear guidelines, suboptimal care will remain the norm.
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Misperceptions like the “glandular version” persist because Sjogren’s medical education is often inaccurate or simply ignored. We need to do a better job for this common, serious disease. Thanks to @DrChadJohr @dysatuonoamia @lupusencycolopedia @SjogrensOrg for your efforts.
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High-prevalence systemic features IMPACT > 50% of Sjogren’s patients
-Arthritis/arthralgia (10% inflammatory)
-Lung disease (many types)
-Neurological features, including SFN, dysautonomia, headache
-Gastrointestinal (many types)
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These high-prevalence features are overlooked when:
-Arthritis is labeled as osteoarthritis, RA, or SLE.
-Lung disease is assumed to be dryness, asthma, deconditioning
-Clinicians don’t know that SFN, dysautonomia, and h/a are common
-GI symptoms are ignored/ called IBS
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In case you were wondering, lung disease >> 10-20% when all patients are screened. ILD is found in up to 65% of asymptomatic patients on HRCT. COPD in 41% (34% of never-smokers). Silent lung disease is still lung disease and should be monitored.
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pubmed.ncbi.nlm.nih.gov/11685093/

These systemic features IMPACT 10-50% of Sjogren’s patients
-lymphadenopathy/lymphoma
-enlarged parotid glands
-kidney
-cutaneous
-hematological (cytopenia, AIHA)
-biological (eg,⬆️IgG, ⬇️Complement})
-constitutional
-Raynaud’s
-Systemic ocular
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Dozens of systemic features IMPACT < 10%.
A few examples:
-myositis
-interstitial cystitis ( ? > 10%)
-pericarditis, myocarditis
-sensorineural hearing loss
-acute or chronic pancreatitis
Common and rare all add up. It defies logic to view Sjogren’s as limited to sicca.
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Still not convinced? Nearly 1000 Sjogren’s patients were evaluated by the ESSDAI, which measures a SUBSET of systemic features. 70-80% of patients had at least one ESSDAI feature at presentation & >90% at 6 years. @ramos_casals
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pubmed.ncbi.nlm.nih.gov/24162151/

SJOGREN’S MYTHS that clinicians tell their patients
-You just have sicca
-SSA neg patients don’t get systemic disease.
-No need to follow unless you have new symptoms
-Sjogren’s does not cause “X” when X is a known Sjogren’s manifestation.
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SJOGREN’S REALITY
-Sjogren’s is always systemic
-Systemic features are often overlooked or misattributed to dryness or psychologized.
-Ongoing monitoring for a variety of systemic features, regardless of symptoms or serostatus, is essential to good Sjogren’s care.
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Failure to diagnose systemic manifestations in earlier stages allows unchecked progression. I know Sjogren’s patients w/ ILD and CKD who developed permanent damage after not being screened for years. Timely diagnosis improves outcomes and quality of life.
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Rheumatologists and medical educators must take the initiative to counter the “ghosting” and misinformation that characterize Sjogren’s medical education. Sjogren’s education needs a serious upgrade at every level, from medical schools to CME.
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Consistent messages are needed to counter widespread misperceptions about Sjogren's. Basic guidelines for monitoring the systemic aspects of Sjogren’s must be developed. Without clear guidance, clinical neglect will continue.
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END NOTE: A large majority w Sjogren’s experience pathologic fatigue, “brain fog”, and/or widespread pain. These profoundly ⬇️QOL. THESE ARE SYSTEMIC FEATURES, possibly neurological, not part of sicca. Serious research is needed, not labels such as FND or fibromyalgia.
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