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Advancements in Sjögren’s Disease

CE / CME

Making Progress in Sjögren’s: Key Considerations for Improving Recognition, Disease Management, and the Potential of Emerging Therapies

Physician Assistants/Physician Associates: 1.00 AAPA Category 1 CME credit

Nurse Practitioners: 1.00 Nursing contact hours, includes 1.00 hour of pharmacotherapy credit

Released: November 21, 2024

Expiration: November 20, 2025

R. Hal Scofield
R. Hal Scofield, MD
CME/CE Information

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Diagnostic Tests for Sjögren’s Syndrome

Early diagnosis is critical for patients with Sjögren’s syndrome, to facilitate early management of symptoms and to prevent more serious complications such as tooth decay, tooth loss, and lymphoma.12-14

However, patients with Sjögren’s syndrome often face diagnostic challenges due to the heterogeneity of the disease. Some may initially present with symptoms similar to those of arthritis, leading to a diagnosis of rheumatoid arthritis or lupus due to a positive ANA or RF, which can delay the correct diagnosis.

Furthermore, many patients do not recognize dryness as a symptom worth mentioning, and primary care physicians may not ask patients about it, resulting in missed or delayed diagnosis. Therefore, primary care physicians should consistently ask about both oral and ocular dryness in patients with suspected autoimmune conditions.12-15

Patients with Sjögren’s disease can be diagnosed definitively through a minor salivary gland biopsy, which is considered the gold standard for diagnosis and part of the research classification criteria. However, this procedure can be difficult to perform and interpret. It requires collaboration among specialists including rheumatologists, oral medicine specialists, ophthalmologists, ear, nose, and throat doctors, and oral pathologists, which can be logistically challenging and may delay diagnosis further.12-14,16

The good news is that patients with severe dry eyes and dry mouth who are positive for anti‑Ro antibodies may not need a salivary gland biopsy for diagnosis. From a clinical perspective, a diagnosis of Sjögren’s syndrome can often be made based on the presence of severe dryness and positive anti‑Ro antibodies. Simple tests like Schirmer’s test for tear production or unstimulated saliva volume measurement can support the diagnosis without the need for invasive biopsy procedures. In fact, many patients with Sjögren’s are diagnosed clinically without a biopsy, which is a widely accepted approach, as long as antibodies and symptoms are present.12-14,16

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Sjögren’s Syndrome Screening Questionnaire

When screening for Sjögren’s in the clinic, I find that the Sjögren’s Syndrome Screening Questionnaire (SSSQ) is a highly useful tool. It has specific questions used to screen for Sjögren’s, which include topics like difficulty eating dry foods such as crackers, dental health, ability to produce tears, and smoking habits.

These questions form the basis of the scoring system, where a score greater than 7 demonstrates good sensitivity and specificity for diagnosing Sjögren’s syndrome. Although the sensitivity and specificity of this questionnaire could still be improved, this approach provides a more reliable method than broad, nonspecific questions and has been validated for detecting Sjögren’s in clinical populations.17

In all, identifying patients who may have Sjögren’s is a matter of asking the right questions. I believe that incorporating these specific questions from the SSSQ into clinical practice will significantly help expedite the diagnosis of Sjögren’s.17

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Atypical Presentations of Sjögren’s

Patients with Sjögren’s can have various atypical presentations. In fact, much like lupus, there may not be a typical presentation for Sjögren’s, as each patient is unique.

Patients with African ancestry, for instance, are more likely to have hypergammaglobulinemia, increased erythrocyte sedimentation rate (ESR), and parotid gland enlargement.

Furthermore, Native American patients exhibit a higher rate of Sjögren’s than the general population, although they tend to have lower rates of abnormal tear and saliva production, but higher disease activity and prevalence.13

Given the diverse ways in which Sjögren’s disease can present, it is critical to think about how different patient populations manifest disease characteristics when diagnosing and managing this condition.13

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Sjögren’s Syndrome Classification Criteria

A diagnosis of Sjögren’s in the clinic should be based on expert opinion and the presence of clinical symptoms, but participation in clinical trials is based on more stringent criteria.

This slide presents the European consensus group criteria for Sjögren’s syndrome. For participation in clinical trials, patients must meet 4 out of the 6 criteria. Of importance, patients must demonstrate the presence of antibodies and salivary gland pathology. 

However, these are classification criteria for research purposes, not diagnostic criteria, although they are useful when considering a diagnosis. As previously mentioned, in cases where patients have severe dryness, positive anti‑Ro antibodies, and other associated features of the disease, a clinical diagnosis may be established without the need for labial salivary gland histopathology. Although these criteria may be required for research purposes, they are not always necessary for clinical diagnosis.14,16

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ACR-EULAR 2016 Classification Criteria for Sjögren’s

In 2016, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) jointly published a new set of classification criteria for Sjögren’s syndrome. These criteria are used for study inclusion and assume that patients already present with dry eyes and dry mouth; therefore, these symptoms are not part of the criteria. Of note, a salivary gland biopsy and anti‑Ro antibodies are valued at 3 points each. To meet the criteria for classification as Sjögren’s syndrome, patients must score 4 points, with at least 1 point coming from either a salivary gland biopsy showing focal lymphocytic infiltrate or a positive anti‑Ro antibody. Unlike previous criteria, anti‑La is excluded, and only anti‑Ro is included.

Although it is important to be familiar with these criteria, they should not be the sole basis for diagnosis. Clinical diagnosis should remain independent of these research classification criteria.18

What’s more important, I believe, is to be familiar with 2 indexes that are used to measure (1) disease activity and (2) symptoms. These are the EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI), which measures involvement of different body systems, and the EULAR Sjögren’s Syndrome Patient-Reported Index (ESSPRI), which measures dryness, pain, and fatigue. Both indexes are common endpoints used in clinical studies of potential Sjögren’s treatments.

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EULAR Sjögren’s Syndrome Disease Activity Index

To measure disease activity, the EULAR developed the ESSDAI, which includes 12 domains. This index has been shown to be highly reproducible, and several of the domains are sensitive to changes in disease status, making it a useful tool to in clinical trials.

In clinical trials evaluating treatments for Sjögren’s, a change in this index often serves as the primary outcome measure. Although this index may also have utility in a clinical setting by providing a numerical measure of disease activity for patients, its primary application is as an endpoint in clinical studies.19

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EULAR Sjögren’s Syndrome Patient-Reported Index

The ESSPRI is a patient‑reported outcome measure that focuses on 3 key symptoms: dryness, pain, and fatigue. It is a simple tool, where patients rate these 3 symptoms over the past 2 weeks using either a visual analog scale or a 0 to 10 scale. This measure provides valuable insight into how patients are feeling, even if their disease activity appears low. Many patients with low disease activity may still report a high symptom burden on this scale.

The ESSPRI can effectively identify patients with high symptom burdens, and in some cases, modified versions of this scale may be used as outcome measures in many clinical trials.20

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