Promoting optimal care for Sjogren's, a common, serious, systemic disease.
INTRODUCTION TO DIAGNOSIS
Sjogren's is a big picture diagnosis
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Key Concepts
Diagnosis typically occurs many years after Sjogren’s symptoms first appear, largely because of lack of clinician training and awareness. Sjogren’s is common, but most patients remain undiagnosed.
Many people with Sjogren’s do not fulfill the Classification Criteria. Classification Criteria are created for research purposes and are necessarily strict. There are no diagnostic criteria for Sjogren’s.
No single test diagnoses Sjogren’s early and accurately. Negative tests do not rule out Sjogren’s. The gold standard for diagnosis is the clinician’s expert opinion.
An estimated 30-40% of people with Sjogren’s are “seronegative”, meaning they don’t carry antibodies such as SS-A. Seronegative patients can still get almost every systemic complication of Sjogren’s.
Sjogren’s is a clinical diagnosis
A clinical diagnosis takes into account the big picture. This includes a detailed medical history, family history, physical examination, and laboratory tests. Symptoms tend to be scattered and vary a lot among patients. Sjogren's is rarely considered as a possible diagnosis by most clinicians; even classic presentations tend to be missed. Many clinicians incorrectly view Sjogren's as an unimportant, mild sicca (dryness) disease. Sjogren's is common, serious, and always systemic.
Sjogren’s symptoms usually come on slowly. Patients often do not mention sicca symptoms to their clinicians, thinking them to be unimportant or a normal part of aging. Yet this could be a good clue to the bigger picture. Clinicians must learn specific questions that could lead them to suspect Sjogren’s. Because few clinicians have adequate Sjogren's training, it is common for patients to be misdiagnosed with fibromyalgia, functional neurological disorders, or ME/CFS.
There are no diagnostic criteria for Sjogren's
While laboratory results can be helpful for diagnosis, normal test results do not rule out Sjogren’s. Some rheumatologists insist that Classification Criteria must be met in order to diagnose Sjogren's. This results in long delays or misdiagnosis, especially for patients who are seronegative (SS-A negative) for "Sjogren’s antibodies". Note that Sjogren's antibodies are not specific to Sjogren's disease.
Classification Criteria are not diagnostic criteria (77). There are no diagnostic criteria for Sjogren’s. Sjogren’s patients who present with prominent systemic features often do not fulfill the Classification Criteria, especially if they are SS-A negative (139). It may take years of progression before they meet these criteria.
"Published classification criteria are designed for use in clinical trials and not for diagnosing patients in a clinic for general management and treatment. As such, they are very strict so that there is absolutely no doubt that a patient has Sjögren’s, something that is important to prove for participating in a clinical trial. Patients that do not meet these criteria can still have Sjögren’s." ~The Sjogren's Foundation
Why is timely diagnosis important?
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Diagnosis is the obvious first step to Sjogren's care. Treatment improves quality of life and can reduce progression and prevent complications.
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A “Sjogren’s knowledgeable” health care team should be assembled as early as possible. At a minimum, this includes a primary care provider (PCP), rheumatologist, ophthalmologist, dentist, and for women, a gynecologist. Other specialists are often needed, such as pulmonologists and neurologists.
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Sjogren’s patients should be routinely monitored for sicca features, systemic manifestations, general comorbidities, and immune system comorbidities.
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Early diagnosis and treatment of systemic Sjogren's disease improves outcomes. Systemic features can be found in almost every patient if you look for them. These include arthritis, lung disease, kidney disease, neurological manifestations, lymphoma, and much more
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It is important to look for immune-mediated diseases that tend occur together with Sjogren’s early on. These often have overlapping features with Sjogren's. Some of the more common ones include thyroid disease, celiac disease, rheumatoid arthritis (RA), spondylitis, and systemic lupus erythematosus (SLE).
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Patients diagnosed with Sjogren’s are at increased risk for cardiovascular disease, osteoporosis, infections and other general comorbidities. PCPs play a key role in prevention and management of these conditions.
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HIV and hepatitis C virus (HCV) can mimic Sjogren’s. These should be ruled out during the process of diagnosis.
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Sjogren's should be ruled out in every person diagnosed with fibromyalgia. Almost every fibromyalgia symptom can be caused directly by Sjogren's.
Read this blog post to learn why the "fibromyalgia" label should usually be dropped once Sjogren's is diagnosed.
Why is Sjogren’s diagnosis so often delayed?
Lack of medical education, misperceptions, and disinterest at all levels in the medical system cause Sjogren’s to be “missed and dismissed.” Few clinicians are trained to recognize even typical Sjogren’s presentations.
Why aren’t there better tests to diagnose Sjogren’s?
This will hopefully change soon. The Sjogren's Foundation supports researchers who are looking for biomarkers specific to Sjogren’s. These novel biomarkers could be game-changers. Being able to diagnose Sjogren’s early and accurately could make a profound difference in our ability to research the disease.
This website addresses Sjogren’s in adults.
Diagnosis is different in the pediatric age group. Children with Sjogren’s often present without complaints of dryness. They often have recurrent swelling and inflammation of the parotid glands. The Sjogren's Foundation has published videos about Sjogren's in children and adolescents.
Updated 03-02-2023