Promoting optimal care for Sjogren's, a common, serious, systemic disease.
MYTHS ABOUT DIAGNOSIS
Key Concepts
This page lists common myths about the diagnosis of Sjogren’s countered with the current understanding of the topic using the best scientific evidence available.
For each myth, you will have resources such as quotes from scientific articles that you can print and take to your clinicians. This information will help you advocate for appropriate medical care.
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To increase your chances of success, please read the SELF-ADVOCACY section: ABOUT SELF-ADVOCACY, KNOW YOUR RHEUMATOLOGIST, and HANDOUTS FOR CLINICIANS before reading this page.
As always, use the 3 P's of self-advocacy when myth-busting:
Prioritize individual myths.
Prepare materials to counter the myth.
Practice what you want to say to your clinician.
Introduction
Despite being a common, serious, systemic disease, it can be challenging to find clinicians who are knowledgeable about Sjogren’s. Sjogren's disease is "notoriously difficult to diagnose", especially in people who do not test positive for SSA. Myths and misperceptions about Sjogren’s remain widespread, even among rheumatologists, the specialists who are responsible for taking the lead on Sjogren’s care. Patients often need to advocate for the diagnosis and management of Sjogren’s and all its manifestations, not just sicca (dryness).
You must be prepared to:
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share reliable information about Sjogren’s from academic sources,
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counter myths about Sjogren's that get in the way of proper diagnosis and care,
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respectfully explain why you are sharing the information and how you think it will help your care.
This page lists common myths about Sjogren’s diagnosis, the current understanding of the topic using the best available scientific evidence, and tools to help you advocate for appropriate medical care.
If you are using this page for the first time, read through the table of contents so you know what is on this page. Rather than trying to read the entire page, try starting with one myth that interests you.
Myths About The Diagnosis Of Sjogren's
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Myth 1: You can confirm that you have Sjogren’s with a single diagnostic test.
Reality: No single diagnostic test can reliably confirm that you have Sjogren’s.
Myth 2: A positive blood test for SSA (anti-Ro antibodies) is required to diagnose Sjogren’s.
Reality: At least one in three people with Sjogren’s does not have a positive blood test for SSA. SSA is just one of the many tests that is used to support a Sjogren’s diagnosis.
Myth 3: A positive blood test for SSB alone (without a positive SSA) does not support a diagnosis of Sjogren’s.
Reality: A positive blood test for SSB alone (without a positive SSA) does support a diagnosis of Sjogren’s.
Myth 4: You must have obvious dry eyes or dry mouth to be diagnosed with Sjogren’s.
Reality: Sjogren’s is a systemic disease that usually, but not always, includes dryness. Sicca, while not always present, encompasses more than just dryness.
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Myth 5: A negative lip biopsy (MSG biopsy) rules out Sjogren’s.
Reality: The lip biopsy can be negative in people with Sjogren’s.
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Myth 6: A normal salivary gland ultrasound (SGUS) rules out Sjogren’s.
Reality: Salivary gland ultrasound can be normal in people with Sjogren’s.
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Myth 7A: You must meet the diagnostic criteria to be diagnosed with Sjogren’s.
Myth 7B: A You must meet the classification criteria to be diagnosed with Sjogren's.
Reality: Many people with Sjogren's do not fulfill the classification criteria.
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Myth 8: Sjogren’s is not important to diagnose because it is a mild disease and there are no treatments for it.
Reality: Sjogren’s is a serious, systemic disease, more than "just sicca".
Other Myth-busting Resources
Use the following key resources with the information about each myth to successfully advocate for better medical care:
Smart Patients - This online community of Sjogren's patients is where you will be able to get tips and support from other patients who have faced similar situations.
MYTHS ABOUT SJOGREN'S - Tools and strategies to counter misperceptions about the fundamental facts of Sjogren's disease.
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KNOW YOUR RHEUMATOLOGIST - It is important to know your rheumatologist’s approach to Sjogren’s to effectively communicate with them.
HANDOUTS FOR CLINICIANS - Learn how to choose and share information about Sjogren’s with your clinician. Simply printing out or emailing them articles or handouts without preparation and explanation is rarely effective.
ABOUT SELF-ADVOCACY - Learn why self-advocacy is necessary and how to be effective at it. The 3 P’s of self-advocacy: Prioritize, Prepare, and Practice, are key to communicating effectively with your clinicians for better medical care.​
Myth 1: You can confirm that you have Sjogren's with a single diagnostic test.
Reality: No single diagnostic test can reliably confirm that you have Sjogren's.
There is no “Sjogren’s test”. Sjogren’s is a big picture diagnosis that takes into account a combination of clinical signs/symptoms, available clinical tests, medical history, and family medical history.
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No single test rules Sjogren’s in or out. Each of the tests commonly used to help diagnose Sjogren’s, the blood test for SSA, minor salivary gland (lip) biopsy, and dryness tests, can be negative (normal) in people with Sjogren’s. These tests can be positive in people that do not have Sjogren’s, including healthy people. Notably, the SSA is frequently positive in other autoimmune rheumatic diseases such as systemic lupus erythematosus. The results of each of these tests can vary over time in people with Sjogren’s, which is why it is not usually recommended to repeat the SSA or the lip biopsy once they are positive, unless you are being tested for a research study.
Sjogren’s is a clinical diagnosis that is supported, but cannot be ruled out, by diagnostic tests.
For more detailed information about the accuracy of tests used for diagnosis, see the Labs For Diagnosis and Minor Salivary Gland Biopsy (Lip Biopsy) pages.
Self-Advocacy Tools
1. Print the Sjogren's Foundation Diagnosis Handout. Circle the entire first paragraph of the handout and highlight the first two sentences, “Sjogren's disease can be difficult to diagnose due to its systemic nature and lack of a single diagnostic test. No diagnostic criteria for Sjogren’s exist for use in the clinical setting.”
See WHY IS IT SO HARD TO GET DIAGNOSED? to learn more.
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2. Print the "Diagnosis" section (p. 17-23) of "Current State of Knowledge on Primary Sjogren's Syndrome, an Autoimmune Exocrinopathy" (153). Highlight these sentences on page 17 of the "Diagnosis" section and share them with your clinician:
"Unfortunately, there is no single diagnostic test to confirm the diagnosis of pSS. Due to its protean and unwillingly insidious presentation, pSS is sometimes difficult to recognize and may delay diagnosis by more than 10 years. Sicca syndrome, fatigue and unspecific musculoskeletal pain can be wrongly taken for manifestations of age, anxio-depression or perimenopause in people with pSS. Systemic manifestations can sometimes precede sicca syndrome, resulting in 'occult pSS'. For these various reasons, the gold standard for individual diagnosis of pSS remains the opinion of an expert clinician."
​The most common reasons you may not get a diagnosis of Sjogren’s even if you have Sjogren’s:
1. you have a negative blood tests for SSA,
2. you have a negative or inadequate lip biopsy (MSGB),
3. you do not have dryness/sicca symptoms,
4. your tests do not show severe dryness.
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Myth 2: A positive blood test for SSA (anti-Ro antibodies) is required to diagnose Sjogren’s.
Reality: At least one in three people with Sjogren’s does not have a positive blood test for SSA. SSA is just one of the many tests that is used to support a Sjogren’s diagnosis.
If a rheumatologist suspects that a patient has Sjogren's based on their signs and symptoms, they will typically order a blood test for SSA as a first step. If the test comes back positive for SSA, they usually arrive at a diagnosis of Sjogren’s. If the test comes back negative for SSA, some rheumatologists do more testing. Others stop the evaluation and do not arrange for other tests that could help them diagnose Sjogren’s.
A negative SSA does not rule out Sjogren’s (6, 79, 164). At least one third of people with Sjogren’s test negative for SSA (they have seronegative Sjogren’s*) so it is inappropriate for a clinician to tell you that you do not have Sjogren’s just because you do not have a positive blood test for SSA (6, 20, 118, 139). Some people who present with systemic features of Sjogren’s, especially neurological ones, are more likely to be SSA-negative.
SSA-negative (seronegative*) Sjogren’s is real and is just as important to diagnose and treat as SSA-positive Sjogren’s.
*seronegative Sjogren's usually refers to SSA-negative Sjogren’s. This is how it is used on Sjogren’s Advocate. Because clinicians use this term inconsistently (see "seronegative Sjogren’s" in the Glossary), you need to ask your clinician what they mean when they use this term.
SSA AND SJOGREN'S goes into detail about the use and misuse of SSA for diagnosing Sjogren’s and why there is so much confusion about this test.
Self-Advocacy Tools
1. Print the Sjogren's Foundation Diagnosis Handout. Highlight the title, and this sentence in the second paragraph, “Approximately 30-40% of patients are negative for anti-SSA/Ro or anti-SSB/La antibodies and are likely to constitute a large portion of the undiagnosed and/or misdiagnosed patient population.”
See WHY IS IT SO HARD TO GET DIAGNOSED? to learn more.
2. Print "I have anti-SSA (Ro) and anti-SSB (La) antibodies. Does it mean I have Sjogren's disease?" from Dr. Brandon Law’s website, Beyond Dryness. Highlight and share these sentences with your clinician:
"Additionally, the absence of these antibodies does not rule out the possibility of Sjogren's Disease. These antibodies can be found in some healthy individuals as well. In order to reach a diagnosis, it is important to note that a positive blood test alone is not sufficient. Additional testing and evaluation by a rheumatologist are necessary."
3. Print out the abstract of "Clinical and serological characteristics of seronegative primary Sjogren’s syndrome: a comparative study" (139) and highlight these sentences:
"The current classification criteria for pSS should not be used in the diagnosis of seronegative pSS, as the agreement between the different sets of criteria was low, and some patients fell outside the classification. Further clinical and laboratory studies are needed to identify the features that distinguish seronegative pSS."
4. If you have neurological features and suspect that you may have Sjogren’s, but your SSA test is negative, print "Neuro-Sjogren: Peripheral Neuropathy With Limb Weakness in Sjogren’s Syndrome." (202) Circle the entire section on page 7 titled, "Diagnostic Tools for Evaluation of Neuro-Sjogren" and highlight these sentences:
"Anti-SSA(Ro)-antibodies were present in only 48% of our patients. The other 52% of patients were diagnosed with Sjogren’s’ syndrome based on sufficient sialadenitis on minor salivary gland biopsy. Antibody status alone is therefore no criterion to rule out Sjogren’s syndrome."
5. DO YOU HAVE ANTIBODIES? and SSA AND SJOGREN'S contain more self-advocacy resources.
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6. Print out this page from the Sjogren's Foundation blog post, The Difficult to Diagnose Sjogren's Patient. Circle the second paragraph and highlight these sentences:
“Up to 40% of patients are not positive for anti-SSA and are seen as “seronegative,” making diagnosis harder.” “And all patients, whether “seropositive” or “seronegative”, are at risk for serious disease and deserve quality care!”
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Myth 3: A positive blood test for SSB alone (without a positive SSA) does not support a diagnosis of Sjogren’s.
Reality: A positive blood test for SSB alone (without a positive SSA) does support a diagnosis of Sjogren’s.
SSB (anti-La) antibodies are found in about 40% (40 of 100) of people diagnosed with Sjogren’s (6, 255). Although SSB was included in earlier Sjogren’s classification criteria, they were eliminated from the current 2016 American/ European classification criteria (4) because SSB usually occurs with SSA in people with Sjogren’s.
More recent data show that isolated SSB (without SSA) occurs in 2-3% (2-3 of 100) of people diagnosed with Sjogren’s and that these patients often have a unique pattern of disease manifestations (113, 255). SSB has a "high diagnostic specificity for SS" (255) and is one of the earliest antibodies to appear.
Self-Advocacy Tools
1. If your clinician will not diagnose you because you have SSB without SSA, print the article, "Systemic phenotype related to primary Sjogren’s in 279 patients carrying isolated anti-La/SSB antibodies" (255).
In the abstract, highlight the conclusion and share it with your clinician:
"Patients carrying isolated La/SSB antibodies represent a very small subset of patients with a systemic SS phenotype characterised by a significant frequency of active patients in most clinical ESSDAI domains but with a relative low frequency of the highest severe organ-specific involvements. Primary SS still remains the best clinical diagnosis for this subset of patients."
2. If your clinician says that SSB is not part of the classification criteria and therefore you do not have Sjogren’s, print "Phenotyping Sjogren’s Syndrome: towards a personalised management of the disease" (113).
On page S-203, highlight and share:
"This small subset of isolated SSB positive patients had a specific clinical and immunological profile that mixed in features characteristic of both immunonegative patients and patients carrying anti-Ro/SS-A antibodies. When considering the practice of medicine, the gold standard for SS clinical diagnosis remains expert opinion as there is no pathognomonic diagnostic test, and we agree with Danda et al. that the best clinical diagnosis for this subset of patients is SS." (113)
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Myth 4: You must have obvious dry eyes or dry mouth to be diagnosed with Sjogren’s.
Reality: Sjogren’s is a systemic disease that usually, but not always, includes dryness. Sicca, while not always present, encompasses more than just dryness.
People with Sjogren’s can develop potentially life-threatening conditions such as interstitial lung disease (241), pulmonary hypertension, and lymphoma (97) long before they develop measurable dryness of the eyes and mouth. When patients present with severe systemic involvement, such as lung disease or lymphoma, clinicians often do not think of Sjogren’s as a possible cause, especially if the patient does not have sicca symptoms. Early diagnosis of Sjogren’s is essential to ensure timely treatment of these severe Sjogren’s manifestations (2, 3, 24).
The systemic manifestations of Sjogren’s most likely to precede sicca are:
- nervous system (neurologic) manifestations, especially small fiber neuropathy and dysautonomia (178, 184),
- skin (dermatologic) manifestations such as annular erythema and vasculitis (3)
- joint swelling, stiffness, and/or pain (arthritis & arthralgia) (3, 214, p. 106)
- Raynaud’s phenomenon (3)
Many clinicians still refer to Sjogren’s as "sicca syndrome" despite undeniable evidence that Sjogren’s is a systemic disease. One out of five Sjogren’s patients (20%) develops systemic (non-sicca) Sjogren’s manifestations before sicca becomes noticeable or measurable (3, 8, 232). Some people with Sjogren’s may never develop sicca (164). People with Sjogren’s without obvious sicca usually experience long delays to diagnosis. Some of them never get diagnosed because most clinicians, including rheumatologists, incorrectly assume that you must have measurable dryness to have Sjogren’s. SICCA is more than just a lack or tears or saliva.
When sicca is present, it provides an important clue that a person may have Sjogren’s. Most clinicians have not been trained to recognize typical presentations of Sjogren’s. They rarely ask about sicca when a patient comes to them with common systemic symptoms of Sjogren’s such as fatigue, cognitive dysfunction ("brain fog"), muscle pain, joint pain, and gastrointestinal pain and motility problems. These symptoms are often misattributed to irritable bowel syndrome, fibromyalgia or psychological conditions, depriving patients of proper diagnosis and management of Sjogren’s.
Don’t Ask, Don’t Tell...
Sicca may be present but may not be recognized.
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Clinicians often do not think to ask about sicca symptoms
and most patients don’t mention sicca symptoms.
Clinicians sometimes assume that patients will tell them about sicca symptoms. Most people with Sjogren’s seek a diagnosis for their systemic (non-sicca) symptoms. Even if they have bothersome sicca, patients rarely volunteer this information (234), thinking it unrelated to their systemic symptoms. One reason why Sjogren's is most often diagnosed in middle-aged women is that they may experience a sudden worsening of sicca around the time of menopause.
Self-Advocacy Tools
1. Print the Sjogren's Foundation Diagnosis Handout. Highlight the first two sentences of the third paragraph: “Classic symptoms for Sjögren’s are dry eyes and dry mouth. However, many patients present with other clinical manifestations first, including pulmonary and neurological involvement, which may also delay Sjögren’s diagnosis.”
The phrase, "many patients present with other clinical manifestations first" is key because it means that people can have Sjogren’s for years before they develop obvious or measurable sicca.
See WHY IS IT SO HARD TO GET DIAGNOSED? to learn more.
2. Print "Not all Sicca is Sjogren’s and not all Sjogren’s is Sicca" (232). On page 2, highlight these sentences in the second paragraph under "Discussion":
"Despite its classic association with dry eyes and dry mouth syndrome, approximately 20% of patients with SS
do not have sicca symptoms. Most of these patients may not ever develop sicca symptoms as part of their
disease manifestations but may have other features of systemic involvement across 12 different disease
domains described in the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI)."
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3. Print: "Sjogren's Without Dryness? Yes, It's a thing"
Please note that this is more common than indicated by the paper because people with non-sicca presentations rarely get diagnosed.
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4. Print the "Diagnosis" section (p. 17-23) of "Current State of Knowledge on Primary Sjogren's Syndrome, an Autoimmune Exocrinopathy" (153). Circle this paragraph on page 17 of the "Diagnosis" section and highlight the sentence that is highlighted here in yellow:
"Unfortunately, there is no single diagnostic test to confirm the diagnosis of pSS. Due to its protean and unwillingly insidious presentation, pSS is sometimes difficult to recognize and may delay diagnosis by more than 10 years. Sicca syndrome, fatigue and unspecific musculoskeletal pain can be wrongly taken for manifestations of age, anxio-depression or perimenopause in people with pSS. Systemic manifestations can sometimes precede sicca syndrome, resulting in 'occult pSS'. For these various reasons, the gold standard for individual diagnosis of pSS remains the opinion of an expert clinician."
5. Ask yourself these SICCA SCREENING QUESTIONS to determine if you are experiencing symptoms of sicca. If you answer yes to any of the questions, even if you don’t feel dry, be sure to point out the symptoms of sicca that you do experience to your clinician. This can help you advocate for getting evaluated for Sjogren’s.
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Myth 5: A negative lip biopsy (MSG biopsy) rules out Sjogren’s.
Reality: The lip biopsy can be negative in people with Sjogren’s.
The lip biopsy (MSG biopsy) is not a perfect test. It is more likely to be positive after many years of disease progression (3, 15, 164). Yet rheumatologists often tell SSA-negative (seronegative) people with a strong clinical picture of Sjogren’s that they do not have the disease. Ignoring the limitations of the current diagnostic tests, including the lip biopsy, leaves large numbers of people with Sjogren’s undiagnosed. The story of my diagnosis illustrates the “wide gap between our understanding of SjS [Sjogren’s] natural course and our ability to diagnose SjS at earlier stages.” (164)
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"A major shortcoming of [the] MSG biopsy in diagnosing SjS is its low reproducibility, and obvious discrepancies exist in the histopathologic evaluation of the MSG biopsy among different institutions or different pathologists." (164)
Despite these limitations, it is usually a good idea to get a lip biopsy or a repeat lip biopsy if you are seronegative and have multiple signs and symptoms of Sjogren’s. A positive lip biopsy opens the door to being taken more seriously and getting more treatments.
Learn more about how the lip biopsy is done.
If you do not have access to a skilled practitioner, or, if your lip biopsy is abnormal but doesn't meet the required focus score, you may need to advocate for a clinical diagnosis of Sjogren's, or to be managed as "possible Sjogren's". You can advocate for yourself by using the articles below that show the limited accuracy of the lip biopsy.
Self-Advocacy Tools
1. Print the abstract of “Clinical and serological characteristics of seronegative primary Sjögren's syndrome: a comparative study” (139) and highlight this sentence:
“The current classification criteria for pSS should not be used in the diagnosis of seronegative patients, as the agreement between the different sets of criteria was low, and some patients fell outside the classification.”
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2. See Myth 7A and Myth 7B below to counter the claim that you must meet the classification criteria for diagnosis. The classification criteria, which require either a positive lip biopsy or SSA, should not be used as a substitute for using clinical judgment when diagnosing Sjogren’s in the routine clinic setting.
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3. Print the article, “Sjogren’s Syndrome without focal lymphocytic infiltration of the salivary glands” (258) and highlight this sentence found in the abstract:
“About 20% (51/229, 22%) of those classified as primary Sjogren’s syndrome had a focus score of zero.”
Point out to your rheumatologist that people with Sjogren’s who are seronegative are stuck between a rock and a hard place when it comes to diagnosis. In studies of SSA-positive Sjogren's patients, lip biopsies are positive about 80% of the time. This means that 20% (1 in 5) of patients with SSA-positive Sjogren's will have a negative lip biopsy, even though they have Sjogren’s. There is no research on the accuracy of lip biopsies in seronegative people with Sjogren’s because, without a positive lip biopsy, many rheumatologists will not diagnose them with Sjogren’s. It is impossible to know if the same percentage of people with Sjogren’s who are SSA-negative will have a negative lip biopsy.
4. A negative focus score (FS < 1, a focus score less than one), accompanied by large numbers of white blood cells (inflammatory infiltrate) on your biopsy, can still support a Sjogren’s diagnosis.
Print the article, "Total area of inflammatory infiltrate and percentage of inflammatory infiltrate identify different clinical-serological better than histopathological parameters" (256), which shows that the presence of many white blood cells (inflammation) can support a Sjogren’s diagnosis, even in the absence of a positive focus score. In the current classification criteria, a focus score of one or greater than one (FS > 1) is considered positive.
Highlight this sentence on page S-198:
“The total area of inflammatory infiltrate and the percentage of inflammatory infiltrate appear significantly higher in patients fulfilling the ACR /EULAR classification criteria for SS, even in those with a FS < 1.”
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Highlight this sentence on page S-201:
“In addition, as it has been suggested in the literature, these two parameters may be useful in overcoming the issue correlated with FS calculation biased by variability in the interpretation of the number of foci or by an insufficient surface area.”
The “two parameters” refer to:
- the total area of inflammatory infiltrate (white blood cells) viewed on the biopsy samples, even if insufficient clusters (foci) are viewed, and,
- the percentage of inflammatory infiltrate (white blood cells), even if insufficient clusters (foci) are viewed.
5. If you have a negative focus score and fibrosis (scarring) on the lip biopsy, you can show that fibrosis supports a Sjogren’s diagnosis. Fibrosis is a sign of long-term damage.
Print the article, “Minor Salivary Gland fibrosis in Sjogren’s syndrome is elevated, associates with focus score and not solely a consequence of aging” (257).
Highlight this sentence on page 8:
“In our analyses, fibrosis distinguishes pSS from those with sicca symptoms who do not meet criteria for SS and performs comparably to [a] biopsy focus score in this regard.”
Highlight these two sentences on page 9:
“In fact, fibrosis was comparable to the predictive power of a biopsy focus score in this analysis.”
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“While the exact cause of SG (salivary gland) fibrosis and its role in SG dysfunction and SS disease remain unknown, the data presented here establish fibrosis as a pathologic feature of SS.”
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Myth 6: A normal salivary gland ultrasound (SGUS) rules out Sjogren’s
Reality: Salivary gland ultrasound can be normal in people with Sjogren’s.
Salivary gland ultrasound (SGUS) is increasingly used as a diagnostic test for Sjogren’s. It is painless, non-invasive, and can be performed in the clinic, a big plus for people who do not want a lip biopsy. A positive SGUS is often considered sufficient to diagnose Sjogren’s in people who have other signs and symptoms of the disease.
A negative SGUS does not rule out Sjogren’s and should prompt further evaluation.
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Limitations of the SGUS
As of 2023, the SGUS is by people in major academic institutions by researchers with special training. Most rheumatologists have not been trained to do this test, but this is changing, and I suspect it will be used more frequently in the near future. Because there are no standard guidelines for interpreting the SGUS at this time, the results may vary based on who is performing the tests.
The sensitivity of the SGUS is slightly lower than the lip biopsy. It is positive in 49-67 % of people with Sjogren’s who were diagnosed (259) using the classification criteria. Like the lip biopsy, SGUS is more likely to be positive after the disease has been progressing for many years.
Importantly, the SGUS is another test for salivary gland damage and function. Because the classification criteria overemphasize the glandular aspects of Sjogren’s, they often fail to detect Sjogren’s in people with systemic (non-sicca) presentations (139). Adding the SGUS to the classification criteria will not remedy this problem.
Self-Advocacy Tools
Print the article “Can salivary gland ultrasonography replace salivary gland biopsy in the diagnosis of Sjogren’s syndrome?” (259).
Highlight this sentence on page 2446:
“The absolute agreement between the ACR-EULAR classification was 80% with a sensitivity of 67% and specificity of 94% and PPV of 92 and NPV of 72%.”
1. If your SGUS is positive, but your clinician still wants a lip biopsy, point out the high specificity (few false positives) and high PPV. In lay terms, a high specificity and high PPV means that a positive SGUS is unlikely to be caused by something other than Sjogren’s.
2. If your SGUS is negative, use the same article and quote above to point out that a negative test does not rule out Sjogren’s because a sensitivity of 67% (at best) means the SGUS missed 1 in 3 people who meet the classification criteria for Sjogren’s, and therefore cannot rule out Sjogren’s.
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Myth 7A: You must meet the diagnostic criteria to be diagnosed with Sjogren’s.
Reality: There are no diagnostic criteria for Sjogren’s. Classification criteria are not diagnostic criteria. The gold standard for Sjogren’s diagnosis is ultimately the clinician’s expert opinion (2, 153, 259).
Note: In Sjogren’s, “systemic” refers to non-sicca manifestations. Learn more about SICCA (which is not just dryness) and the difference between Signs, Symptoms, and Manifestations to better understand this section.
This myth is best read together with Myth 7B, immediately below.
1. There are no diagnostic criteria for Sjogren’s.
Diagnostic criteria are, “a set of signs, symptoms and tests for use in routine clinical care to guide the care of individual patients”. (77) The American College of Rheumatology (ACR) no longer endorses diagnostic criteria for rheumatic diseases such as Sjogren’s, systemic lupus, or rheumatoid arthritis. There is no single test or even set of tests that can rule in or rule out most rheumatic diseases, especially one as varied as Sjogren’s.
https://rheumatology.org/criteria
2. The Sjogren’s’ classification criteria are often misused as diagnostic criteria.
If a rheumatologist tells you that you cannot have Sjogren’s because you “don’t meet the criteria” they are confused about the purpose and limitations of the classification criteria and are misusing them as diagnostic criteria (which do not exist).
The Sjogren’s classification criteria are a well-defined but narrow/limited set of features of Sjogren’s,
“well-suited as criteria for enrollment in clinical trials” (4). They are not intended to diagnose every person with Sjogren’s.
Using the classification criteria to rule out Sjogren’s misses many people with Sjogren’s who have signs and symptoms of the disease other than those measured by the classification criteria. If the classification criteria are not met, further evaluation is often needed to diagnose Sjogren’s.
See Myth 7B, immediately below, for more information.
The gold standard for Sjogren’s diagnosis is ultimately the clinician’s expert opinion (2, 153, 269).
Sjogren’s should be a big picture diagnosis.
Diagnosing Sjogren’s should not be reduced to checking off boxes on a list of classification criteria.
Self-Advocacy Tools
To make the point that the classification criteria are not sufficient for diagnosis, print and highlight these articles:
1. Sjogren's Foundation Diagnosis Handout
Circle the entire first paragraph of the handout and highlight the sentence, “No diagnostic criteria for Sjogren’s exist for use in the clinical setting.”
See WHY IS IT SO HARD TO GET DIAGNOSED? to learn more.
2. “Can salivary gland ultrasonography replace salivary gland biopsy in the diagnosis of Sjogren’s syndrome?” (259)
Highlight these sentences in the introduction:
"The purpose of the classification criteria is to assist in defining homogeneous study groups for clinical research. In daily clinical practice however, these criteria are often helpful for diagnostic purposes, but expert opinion should be leading the final clinical diagnosis."
3. “Distinctions Between Diagnostic and Classification Criteria” (77)
On page 9, circle the entire conclusion and highlight the final sentence:
"The process of diagnosis, particularly for complicated multisystem involvement typical of rheumatic diseases, is a highly complex cognitive process that requires synthesis of many data points, typically beyond a simple algorithm-based set of criteria."
A Sjogren’s diagnosis should not be reduced to a box-ticking exercise.
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Myth 7B: You must meet the classification criteria to be diagnosed with Sjogren’s.
Reality: Many people with Sjogren’s do not fulfill the classification criteria.
Note: In Sjogren’s, “systemic” refers to non-sicca manifestations. Learn more about SICCA (which is not just dryness) and the difference between Signs, Symptoms, and Manifestations to better understand this section. This myth is best read together with Myth 7A, immediately above.
The classification criteria are a research tool. They purposely exclude some people with Sjogren’s.
The classification criteria are “primarily intended to create well-defined relatively homogenous cohorts for clinical research; they are not intended to capture the entire universe of possible patients.” (77) The classification criteria should not be misused as diagnostic criteria. There are no diagnostic criteria for Sjogren’s.
The classification criteria are inadequate for diagnosing Sjogren’s.
The classification criteria focus almost entirely on the glandular (sicca) aspects of Sjogren’s. The 20% or more who present with systemic (non-sicca) manifestations often do not meet the classification criteria (42, 139, 232). Even people with classic sicca symptoms may not meet the classification criteria for many years, especially if they are SSA-negative, because it takes a long time for Sjogren’s to cause enough damage for the sicca tests or the lip biopsy to be positive (164). It is likely that many people with Sjogren’s are never diagnosed.
A lack of measurable sicca and glandular damage do not rule out Sjogren’s.
Other than SSA, each test used in the current 2016 ACR/EULAR classification criteria Link to CC page is an objective measurement of glandular damage or function. The classification criteria do not include systemic manifestations even though evidence clearly shows that Sjogren’s is a multisystem disease, never limited to the salivary and tear glands (sicca).
People who present with systemic manifestations are a high-risk group that should be a priority for timely diagnosis and treatment (3). Because a “large number of non-sicca features might appear up to 20 years before development of sicca symptoms” (42), people with systemic presentations often experience extreme delays to diagnosis.
Sjogren’s is a multisystem disease and more than “just sicca”.
It is time to re-evaluate and update the classification criteria to better reflect the reality of Sjogren’s.
Each test used in the classification criteria has limited accuracy and can produce both false positive and false negative results.
The SSA test is negative in 30-50% of people with Sjogren’s (see Myth 2 above).
The lip biopsy is negative in 20%, possibly more, of people with Sjogren’s (see Myth 5 above).
The sicca test results: ocular staining score, Schirmer’s test, and unstimulated salivary flow test vary by time of day, systemic and topical mediations/treatments, environmental conditions, and variations in technique and interpretation. See classification criteria to learn more about the purpose and limitations of these tests.
The scoring system for the Sjogren’s classification criteria is designed such that either the SSA, the lip biopsy, or both must be positive to meet the criteria.
The scoring system is problematic for SSA-negative patients because the lip biopsy can be negative if it is performed too early or too late. There is no way to know what too early or too late is in an individual patient. Patients do not always have access to skilled practitioners, and some are not willing to undergo the procedure. Learn how limitations of lip biopsy accuracy contributed to my own extreme delay to diagnosis in the blog post, 12 Reasons Why Patients Don’t Get Diagnosed.
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Sjogren’s is a “big picture” diagnosis that considers medical history, family history, systemic signs and symptoms, and physical examination. None of these important data points are part of the classification criteria.
At least 20% of people with Sjogren’s first present without sicca (8, 61, 232). These patients are often misdiagnosed with systemic lupus erythematosus, rheumatoid arthritis (23), fibromyalgia, or functional disorders, and may never be correctly diagnosed with Sjogren’s.
People with Sjogren’s neurological manifestations are especially likely to present without sicca (202, 232). Neurological disorders, like most systemic manifestations, are not caused by an attack on the exocrine glands (214, pp.118-119). This is why Sjogren’s should never be called a “glandular disease”, “sicca syndrome” or an “autoimmune exocrinopathy”.
Sjogren’s is a clinical diagnosis that is supported by, but cannot be ruled out by, current diagnostic tests.
“Until we have better tests, ‘the gold standard’ for diagnosis still remains the clinician’s expert opinion” (2)
~ Sjogren’s expert, Dr. Fredrick Vivino.
Unfortunately, there are few experts.
Self-Advocacy Tools
1. Print the Sjogren's Foundation Diagnosis Handout. Circle the entire first paragraph of the handout and highlight the last sentence: "Many patients also do not fulfill the classification criteria that are very stringent and meant only for use in clinical trials—so when these criteria are used for everyone, a Sjogren’s diagnosis is often missed."
See WHY IS IT SO HARD TO GET DIAGNOSED? to learn more.
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2. Your rheumatologist tells you that you cannot have Sjogren’s because you don’t meet the classification criteria for Sjogren’s. Print the “Diagnosing Sjogren’s” page from the Sjogren’s Foundation.
Highlight these sentences in the third paragraph:
“This criterion was developed for Sjögren’s patient entering clinical trials and not for diagnosing patients in a clinic. Thus, diagnosis of Sjögren’s can sometimes be an 'art' based on the physician’s assessment of symptoms plus objective tests.”
Highlight this sentence in the fourth paragraph:
“For a patient to be included in a clinical trial, diagnosis for any disease must meet very strict criteria. These criteria are called 'Classification Criteria' and are NOT designed for diagnosis in the clinician’s office for management and treatment.”
3. Your rheumatologist tells you that you don’t meet the “criteria” or the “diagnostic criteria” for Sjogren’s. Show them why the classification criteria should not be misused as diagnostic criteria.
Print the article, “Distinctions Between Diagnostic and Classification Criteria?" (77)
Note: This paper is a gold mine of quotes. It is posted on the American College of Rheumatology (ACR) website to show rheumatologists the purpose and limitations of classification criteria. Emphasize that this paper is highlighted on the ACR website to make the point that classification criteria should not be misused as diagnostic criteria.
On page 2, highlight, “Diagnostic criteria are a set of signs, symptoms, and test for use in routine clinical care to guide the care of individual patients.”
and
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“Classification criteria are standardized definitions that are primarily intended to create well-defined, relatively homogeneous cohorts for clinical research: they are not intended to capture the entire universe of possible patients…”.
On page 3, highlight, “This may make classification criteria inappropriate for use in routine clinical care.”
On page 9, circle the “Conclusion” section and highlight the last sentence:
“The process of diagnosis, particularly for complicated multisystem involvement typical for rheumatic diseases, is a highly complex cognitive process that requires synthesis of many data points, typically beyond a simple algorithm-based set of criteria.”
4. If you have neuropathy (many types) and other signs and symptoms of Sjogren’s but do not satisfy Sjogren’s classification criteria, print “Neuro-Sjogren’s: Peripheral Neuropathy with Limb Weakness in Sjogren’s Syndrome” (202).
On page 6, highlight, "Neuropathy, however often preceded the development of sicca syndrome, rendering the recognition of Sjogren’s syndrome during early stages of the disease course nearly impossible."
On page 7, highlight, "Anti-SSA (Ro-antibodies) were present in only 48% of our patients” and “Antibody status alone is therefore no criterion to rule out Sjogren’s syndrome."
5. For more self-advocacy tools, see the other Myths About Diagnosis on this page.
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Myth 8: Sjogren’s is not important to diagnose because it is a mild disease and there are no treatments for it.
Reality: Sjogren’s is a serious, systemic disease, more than "just sicca".
Treatments often improve quality of life, and may reduce progression. Early treatment of organ system manifestations such as Sjogren’s lung disease and kidney disease reduces progression and improves outcomes (54, 89,132)
There are many available treatments for Sjogren’s. Please refer your clinician to the Sjogren’s Foundation clinical practice guidelines and Self-Advocacy Tool #3 below to learn more about treatment options.
Self-Advocacy Tools
1. Print the Sjogren's Foundation Diagnosis Handout.
In the 3rd paragraph, highlight the last sentence, “Sjogren’s is a serious systemic disease that can have serious complications, so an early diagnosis is important.”
See WHY IS IT SO HARD TO GET DIAGNOSED?​ to learn more.
2. See User Guide for Handout: Sjogren’s is More Than “Just Sicca” for a detailed self-advocacy tool that demonstrates that Sjogren’s is a serious, systemic disease.
3. Print the Sjogren’s Foundation Clinical Practice Guidelines Systemic Manifestations in Sjogren's Patients and show your clinician(s) the treatment recommendations for Sjogren’s. Let them know that more clinical practice guidelines are available here on the Sjogren's Foundation website.
4. For more self advocacy tools regarding treatment recommendations, see Myth10 on the Myths About Sjogren's page.
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Updated 04-04-2024