INTRODUCTION TO DIAGNOSIS
Key Concepts
Sjogren's is a big picture diagnosis.
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.
Sjogren's is important to diagnose. Timely diagnosis and management can reduce progression, improve quality of life, and detect systemic manifestations earlier when they are more likely to respond to treatment.
​
Many people with Sjogren’s do not fulfill the Sjogren's classification criteria. Classification criteria are a research tool that exclude many people with Sjogren's and should never be used as a substitute for clinical judgment. They are helpful for diagnosis when they are positive.
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 test positive for typical autoantibodies such as SSA.
​
Self-advocacy tools help many people with Sjogren's work with their clinicians to get a diagnosis.
See the PCPs Steps to Diagnosis page to learn about the signs and symptoms that should prompt an evaluation for Sjogren's.
Sjogren’s is a clinical diagnosis that is supported by,
but CANNOT be ruled out by, diagnostic testing.
“Until we have better tests, 'the gold standard' for diagnosis still remains
the clinician’s expert opinion"
~Sjogren's expert Dr. Fredrick Vivino (2)
Sjogren’s is a clinical diagnosis.
​
A clinical diagnosis takes the big picture into account. 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; most people remain undiagnosed because even classic presentations are often missed. Many clinicians incorrectly view Sjogren's as an unimportant, mild sicca (dryness) disease. Sjogren's is never just dryness; it is serious and always systemic.
Sjogren’s symptoms usually come on slowly. Patients who have sicca (dryness)symptoms may not notice or report them 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 (263).
When people present with sicca, "fibromyalgia" symptoms, or "red flags", clinicians should ask about sicca and other Sjogren's features. Learn more about how to recognize Sjogren's and the first steps to diagnosis.
Because few clinicians have adequate Sjogren's training, it is common for Sjogren's symptoms to be misattributed to psychological disorders.
Many people who do have Sjogren's do not fulfill the classification criteria.
Classification criteria are not diagnostic criteria (77). They are often misused as diagnostic criteria. There are no diagnostic criteria for Sjogren’s.
While laboratory results can be useful 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 (SSA-negative) for "Sjogren’s antibodies". Note that Sjogren's antibodies are not specific to Sjogren's disease. See the glossary to learn more about the various ways that clinicians define "seronegative".
An estimated 30-40% of people with Sjogren’s are “seronegative”, meaning they don’t test positive for typical autoantibodies such as SSA. No single test diagnoses Sjogren’s early and accurately. Negative tests do not rule out Sjogren’s. Seronegative Sjogren's disease "remains an important diagnosis not to overlook since these patients are also at risk of potentially serious extra-glandular manifestations, including lymphoma" (263).
Sjogren’s patients who present with prominent systemic features often do not fulfill the classification criteria, especially if they are SSA-negative (139). It may take years of progression (if ever) 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?
​
-
Diagnosis is the first step to Sjogren's care. Treatment improves quality of life and can reduce progression and prevent complications. "Because of this significant risk for malignancy and potentially serious organ-threatening extraglandular manifestations, it remains vital to accurately diagnose and monitor patients for these complications." (263)
-
A Sjogren’s-knowledgeable health care team should be assembled as early as possible. At a minimum, this includes a primary care practitioner (PCP), rheumatologist, ophthalmologist, dentist, and for women, a gynecologist. Other specialists are often needed, especially pulmonologists, neurologists, gastroenterologists, and ear, nose and throat doctors.
-
Every person with Sjogren's should be monitored for sicca features, systemic manifestations, and comorbidities, regardless of symptoms or antibody status.
-
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.
. -
It is important to look for immune-mediated diseases that often co-occur with Sjogren’s early on and over time. 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).
-
Patients diagnosed with Sjogren’s are at increased risk for cardiovascular disease, osteoporosis, infections and other general comorbidities. Primary care clinicians play a key role in prevention and management of these conditions.
-
HIV and hepatitis C virus (HCV) can mimic Sjogren’s. These should be ruled out during the process of diagnosis.
-
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.
​​
PCP STEPS TO DIAGNOSIS outlines the steps needed to arrive at a diagnosis of Sjogren's.
For more information and self-advocacy tools:
Self-Advocacy for Diagnosis
Myths About Diagnosis
Why is Sjogren's so hard to diagnose?
12 Reasons People With Sjogren's Don't Get Diagnosed.
​
If you don't have SSA or SSB: See SSA and Sjogren's for more self-advocacy tools.
Diagnosis of Sjogren's is different in children.
​​
Children with Sjogren’s often present without complaints of dryness.
​
They often have recurrent swelling and inflammation of the parotid glands.
​
Please visit the Sjogren's in Children section of the Sjogren's Foundation website.
Updated 08-20-2024