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Sjogrens Disease What HCPs Must Know
Current State of Sjögren’s Disease: What HCPs Must Know

Released: January 26, 2026

Nancy Carteron
Nancy Carteron, MD, FACR
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Key Takeaways
  • Diagnosing Sjögren’s disease early can be challenging in practice, but guidelines exist to address the many manifestations of this systemic autoimmune disease.
  • Several biomarkers like C-reactive protein, complement levels, IgG, free light chains, and autoantibodies are available to help healthcare professionals classify the state and severity of Sjögren’s disease.
  • Interdisciplinary care is critical to improving patient outcomes, and these teams often include rheumatology, eye care professionals, dental/oral medicine, and hematology healthcare professionals, among others. 

The concept of phenotyping in Sjögren's disease is evolving right now. Historically, this disease has been thought of as glandular vs systemic. In my approach to classifying Sjögren’s disease, I start with a broad patient overview as I would with anyone suspected of having an autoimmune disease. That includes taking a detailed history, where I ask patients, “When were you last well? And what has happened since?” I want to get an idea of each patient’s health experience over time, and what factors trigger or improve symptoms. Asking these questions will let you know if patients might have Sjögren’s disease and guide testing to confirm the diagnosis. Often, the phenotype will consist of glandular dysfunction with dryness, joint pain, fatigue/cognitive dysfunction, and maybe parotid or submandibular enlargement. Although these symptoms often fluctuate, they will help you determine the predominant type of disease patients may have over time.

Next, I consider what the patient’s primary limitation is. For example, neurologic symptoms, fatigue, ocular or oral dryness, or if they have some symptoms of lung involvement that may need to be worked up. Then I develop a rough draft of how I would classify their disease phenotype and severity and confirm if I think it is Sjögren's alone or associated with another autoimmune disease such as systemic lupus erythematosus or rheumatoid arthritis. With this, I ensure to ask the necessary questions and further investigate the many organs that can be involved in Sjögren's disease. For example, if patients have a long history of a nonproductive cough, have they seen a pulmonologist and/or had pulmonary function tests or chest imaging?

Others might ask their patients questions focusing on symptoms of fatigue, dryness and pain, and other questions to determine which organs might be involved. Is it important to determine the significance and severity of the involvement? Is it a minor annoyance, or is it something that needs further investigation? Healthcare professionals (HCPs) need to be aware of the full spectrum of what can be involved in Sjögren’s and how the disease can present so the diagnosis is not missed.

Guidelines and Resources for Sjögren’s Disease
As I mentioned above, there remains a lack of awareness of the full spectrum of Sjögren’s disease. However, there are several evidence-based clinical guidelines that address various aspects of the disease. For example, the Sjögren’s Foundation, European League Against Rheumatism, British Society for Rheumatology, Brazilian Society of Rheumatology, and Japanese Society for Sjögren’s Syndrome, among others, have published their guidelines or recommendations for diagnosing and managing this disease.

In the US, the Sjögren’s Foundation has driven the initiative for developing clinical practice guidelines and, in doing so, has brought other professional organizations from different medical specialties together to create consensus. The first guideline from 2017 focused on the use of biologics, musculoskeletal, and fatigue. The foundation has since supported development of guidelines for pulmonary manifestations of Sjögren’s and neurologic manifestations, including peripheral neuropathy, mononeuropathy, and autonomic neuropathy. In addition, there are planned guidelines for other manifestations (ie, central nervous system, lymphoma/myeloma/amyloid, vasculitis). The current project at the Sjögren's Foundation is the development of guidelines for central nervous system manifestations of Sjögren’s disease, and involves rheumatology, neurology, psychiatry, and sleep medicine specialists. Evidence-based guidelines take time, effort, and resources to be done effectively.

There are additional resources that HCPs can also access in the meantime. The Sjögren's Foundation has a professional newsletter called Sjögren's Quarterly. You can sign up for this free of charge, and I have found the newsletter to be one of the best ways to stay updated on what is happening in Sjögren’s globally. The foundation also provides continuing medical and professional education opportunities for HCPs through online modules, conferences, and seminars. SjogrensAdvocate.com is another resource that helps HCPs better understand Sjögren’s disease and can help patients access support. Next, a group of rheumatologists, ophthalmologists, optometrists, dentists, and researchers meets monthly in an informal group called Sjö-Net. In these meetings, HCPs discuss various topics and tackle some of the issues in diagnosing and managing Sjögren’s disease.
Any HCP can join; you just have to submit your name and email to receive the information regarding these meetings.

Novel Therapies and Biomarkers: What Lies Ahead
There is also a lack of consensus among rheumatology and other Sjögren's specialists in terms of integrating new and emerging therapies in everyday practice. My approach is shaped by my initial experience as a bench researcher in molecular virology and cellular immunology. When I think of autoimmune diseases, in general, I think of the underlying  mechanism of the disease. I consider the phenotype and what is causing it. Which part of the immune or neurologic system is dysregulated? You can form a hypothesis based on this information. I take a scientific approach, as opposed to relying on pattern recognition or a diagnostic algorithm. This has been effective over time in working with complicated, multisystem autoimmune patients, like those with Sjögren’s.

I also ask: What are the unmet needs? What gaps in care are still present? Has the patient had their eye care addressed? Has their oral medicine care been addressed? Has their OB/GYN addressed potential specific issues? From a strictly internal medicine and immunology perspective, I assess whether patients are primarily exhibiting more of an early stage of immune system dysfunction (innate immunity).  At this stage, patients may experience severe fatigue and joint pain whereas patients with the disease present for some time likely have more adaptive immune dysfunction (eg, T- and B-cells). When the immune response is continually driven, researchers think this pushes the formation of germinal centers in the target tissue, thus predisposing them to lymphoma or multiple myeloma.

Of note, biomarkers and other clinical clues are going to vary depending on the patient. There are a few that most HCPs should be comfortable identifying and interpreting. The first comprises several inflammatory markers, including elevated erythrocyte sedimentation rate and or C-reactive protein, supporting an inflammatory phenotype. Many patients also develop positive autoantibodies, SSA (Ro 52, Ro 60), SSB, ANA (speckled IFA pattern), and rheumatoid factor.  However, 30% to 40% of patients with Sjögren's (minor salivary gland positive) do not have Ro/SSA or La/SSB autoantibodies present. This patient population is more challenging for HCPs to identify as Sjögren’s disease without further investigation, often including a minor salivary gland biopsy (lower lip).

If an immune-complex disease is active process, complement levels decrease. So HCPs can assess C3 and C4 complement levels. Finally, there is a good set of clinical criteria to identify those at higher risk for developing lymphoma due to Sjögren's disease. For example, patients with high focus scores, 4 or greater, on lip biopsy have a higher risk of developing lymphoma. Increased standardized uptake values on PET/CT (>3.0), and vasculitis are other risk factors.

The Importance of Interdisciplinary Care
The final component of delivering effective care to patients with Sjögren’s disease is taking a multidisciplinary approach. Yet the members who make up this team often come down to what expertise is in your area and the HCPs whom you trust. That is because any organ in the body can be involved, so you are going to have patients with different manifestations who are going to need to see different specialists.

Core members of this team comprise your eye care HCPs: ophthalmology and optometry. They will treat patients’ dry eyes and/or vision changes and help prevent ocular surface damage. Because there is such a focus on dry eyes currently, most will have a dry-eye specialist in their area, even if there is not a Sjögren’s eye care specialist. In the context of clinical trials, many of the classification criteria that are currently used include validated ocular measurements like the ocular staining score and Schirmer’s test. So having somebody on the team who is an expert and knowledgeable in using these is valuable. These criteria can help assess disease severity and be used to measure improvement in outcomes with the use of targeted therapies, as well. Next, patients with Sjögren’s disease can experience oral or dental damage. This directly affects patients’ quality of life and can be a huge financial burden as well. Therefore, dental and oral medicine specialists make up key members of the team.

It is important that patients see rheumatology HCPs because they can ensure patients know how to prevent certain issues/symptoms. A problem persists here, though, because many rheumatology HCPs may not be aware that they play a critical role in the prevention of disease progression or damage. Within the internal medicine space, not every patient will have to see other subspecialists because rheumatologists also are trained as internists and we can manage a whole spectrum of issues ourselves. The things that we need extra expertise in include when lung involvement (eg, interstitial lung disease) is present, so pulmonology is needed. Then neurology must be included when patients experience significant nervous system-related disease (ie, ganglionopathy). Finally, hematology HCPs may be required for those at high risk of developing lymphoma or other hematologic malignancies. As you can see, there is no one-size-fits-all approach to managing Sjögren’s disease. If you do not have these specialties available in your area, you can always access additional resources online or reach out to those outside of your state.
 
The bottom line is that patients may be seeing 10 other specialties before they reach rheumatology. Therefore, we need somebody who is knowledgeable on Sjögren’s disease and can promptly develop treatment plans with patients. There is so much information out there right now, so it is just a matter of developing your expertise, keeping an open perspective of what the whole spectrum of the disease can be, and minimizing the disease impact where possible.

Your Thoughts
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