Commonly characterized by dry eyes and mouth, Sjögren’s disease is a systemic autoimmune disease that can affect up to 15% of those diagnosed with lupus.  Keep reading to learn more about this complex condition and how it overlaps with SLE.

• Introduction
• Causes of Sjögren’s Disease
• Sjögren’s Syndrome as an Overlap of SLE
• Symptoms of Sjögren’s Disease
• Diagnosing Sjögren’s Disease
• Treating & Managing Sjögren’s Disease
• Prognosis & Sjögren’s Disease Complications
• In Conclusion

What is Sjögren’s disease?

Sjögren’s disease is one of the most prevalent of the autoimmune diseases along with systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and scleroderma. Though Sjögren’s may initially present in the mouth and eyes, it can progress to being “systemic” and affect the joints, heart, nervous system, kidneys and lungs,. In rare cases, Sjögren’s may even lead to the development of non-Hodgkin lymphoma.

Sjögren’s disease was named after Swedish ophthalmologist Dr. Henrik Sjögren in the early part of the 20^th century – though symptoms of the disease can be traced much farther back in history, Dr. Sjögren was the first to see the connection between the “triad” of distinct symptoms: polyarthritis (arthritis affected at least five joints), xerostomia (dry mouth) and keratoconjunctivitis (inflammation of the cornea and conjunctiva of the eye) which he found occurring mainly in women. Dr. Sjögren’s published work received a cold welcome in Sweden. It was not until 1943 when his work was published in English did Dr. Sjögren receive the recognition he deserved.

There are two types of Sjögren’s disease:

• Primary Sjögren’s disease is diagnosed when there are no other conditions such as autoimmune diseases present.
• Secondary Sjögren’s disease is diagnosed when an individual has other autoimmune diseases such as RA or SLE. The symptoms of secondary Sjögren’s are typically milder than primary Sjögren’s symptoms. In the presence of another autoimmune disease, secondary Sjögren’s is considered an overlap disease.

Globally, Sjögren’s disease – both primary and secondary – affect about 0.4% of the population with the largest rate of incidence in Europe. In the United States alone, approximately 4 million have Sjögren’s syndrome while another 2.5 million may have it, but have yet to be properly diagnosed. 90% of individuals with Sjögren’s syndrome are middle-aged women though the disease can affect anyone of any gender, age and race. An individual may have symptoms for up to three years before Sjögren’s is diagnosed. The rate of incidence seems to increase with age. Proper diagnosis and swift treatment are imperative in order to maintain a good quality of life.

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Sjögren’s Disease as an Overlap of SLE

Primary Sjögren’s and SLE share the most common characteristics of all the connective tissue diseases and therefore can overlap. A 2012 literature review estimates that as many as 15% of individuals with SLE will develop symptoms of Sjögren’s in their lifetime.

Individuals with SLE who also have Sjögren’s have more immunological markers or antibodies including rheumatoid factors (RF), anti-Ro/SSA and anti-La/SSB than individuals with SLE alone. The researchers of the 2012 review also discovered individuals with SLE who also have Sjögren’s have milder SLE symptoms and more predominant Sjögren’s symptoms. Those who have Sjögren’s as an overlap of SLE experience more skin symptoms including malar rash, photosensitivity and Raynaud’s phenomenon. Individuals with both SLE and Sjogren’s report a higher incidence of fatigue and low blood platelet count (thrombocytopenia).

Treatment for both may include hydroxychloroquine, immunosuppressants and corticosteroids. As with any connective tissue diseases that overlap, individuals will receive treatment plans based on their unique symptomatic profile.

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Causes of Sjögren’s Disease

It is unclear what specifically causes Sjögren’s. Researchers believe there may be a combination of factors that put certain individuals at risk. A review of the existing literature suggests these factors:

• Genetics, including specific haplotypes or DNA variations;
• Environmental factors and infectious agents including hepatitis C, Epstein-Barr virus and human T-cell leukemia virus;
• Hormone levels such as a decrease in estrogen;
• Epithelial cell function in the salivary glands and lacrimal glands around they eyes;
• Increased levels of dendritic cells and interferon-alpha (IFN-a) found in saliva;
• Infiltration of T-lymphocytes into the salivary and lacrimal glands which may kill epithelial cells;
• Cytokines, though it has not yet been determined which ones; and/or
• Increased levels of B-cell activating factor (BAFF);

Autoantibodies may also play a role in triggering Sjögren’s. Nair and Singh note that the autoantibody called anti-SSA/Ro is present in individuals with Sjögren’s, though it is not clear how this would work. Clearly, more research needs to be done in order to develop more effective treatments.  Researchers of a 2017 study maintained that “new clinical and laboratory biomarkers are needed to improve the diagnosis of [Sjögren’s disease], to categorize subsets of patients, and to unmask pathogenic mechanisms which may represent novel therapeutic targets.”

Symptoms of Sjögren’s Syndrome

Symptoms of Sjögren’s can vary from person to person, but the most common initial symptoms are dry, burning and itchy eyes and dry mouth. These conditions can put the individual at risk for developing eye infections, gum inflammation, oral yeast infections and pain as the saliva glands around the face swell.

Other symptoms may include:

• joint swelling, stiffness and pain;
• swollen salivary glands behind the jaw;
• skin rashes and dryness;
• vaginal dryness;
• persistent dry cough;
• fatigue;
• neurological problems including cognitive dysfunction or brain fog;
• dry nose, lips and throat;
• chronic sinusitis and nosebleeds;
• changes in taste and smell;
• acid reflux, esophagitis and heartburn;
• difficulties swallowing;
• chronic bronchitis or pneumonia;
• interstitial lung disease;
• shortness of breath;
• eye infections;
• irritable bowel and nausea;
• photosensitivity;
• vasculitis; and
• autoimmune pancreatitis;

Everyone experiences these symptoms differently, which often makes it difficult to gauge whether they are serious or not. It is therefore important to always report any new symptoms that are experienced, no matter how inconsequential they may seem. Seeking the care of a trusted healthcare practitioner can help eliminate speculation and fear while working to treat any underlying conditions.

As with conditions like lupus and RA, Sjögren’s symptoms can flare from time to time. Neil I. Stahl, M.D. writes that when an individual experiences a Sjögren’s flare, they typically report increased dryness of the eyes and/or mouth, joint discomfort and extreme fatigue. A healthcare practitioner should work with the individual to make sure the symptoms are truly the result of a flare and not another underlying condition.

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Diagnosing Sjögren’s Disease

A rheumatologist may initially diagnose Sjögren’s disease, but the symptoms may be diagnosed by other specialists, including dentists, ophthalmologists and other practitioners who are experienced with Sjögren’s.  A conclusive diagnosis can be difficult as there is not one, definitive test that is determinative on its own.  Instead a myriad of tests need to be performed. Diagnosing Sjögren’s may include:

• completing a health history assessment to determine any previously diagnosed conditions, family history of autoimmune disease, medications currently prescribed, lifestyle and current symptoms;
• completing a physical exam from head to toe to determine severity of symptoms;
• ordering blood and urine tests to look for specific antibody profiles often present in Sjögren’s and an antinuclear antibody (ANA) test to check for autoimmune disorder;
• Schirmer’s test, which checks tear gland function to make sure they are keeping eyes moist;
• ocular surface staining to check dryness on the surface of eyes;
• salivary gland scans to look for inflammation and swelling;
• kidney and liver function tests;
• lip biopsies to look for inflammation of the glands that lead to mouth and eye dryness;
• sialometry tests, which measure how much saliva is being produced; and
• ultrasonography that looks structurally at the salivary glands for any characteristic changes that may be occurring.

In 2016, the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) partnered to create the Sjögren’s disease classification criteria. This criteria was developed to help clinicians diagnose Sjögren’s based on a scoring system.  Using the chart below, a clinician would add the scores, and after eliminating certain exclusionary factors, a score of 4 or more would  indicate primary Sjögren’s disease.

 

Item	Weight/Score
Labial salivary gland with focal lymphocytic sialadenitis and a focus score of ≥1 focus/4 mm2	3
Anti-SSA/Ro positive	3
Ocular Staining Score ≥5 (or van Bijsterveld score ≥4) in at least 1 eye	1
Schirmer’s test ≤5 mm/5 minutes in at least 1 eye	1
Unstimulated whole saliva flow rate ≤0.1 ml/minute	1

 

A rheumatologist trying to diagnose Sjögren’s will also look at how frequently symptoms are occurring and how troublesome they are. He or she will also review an individual’s health, what kind of medications are currently being taken, the ocular staining score and unstimulated whole saliva flow rate measurements.

Treating & Managing Sjogren’s Disease

An individual’s symptoms will determine a course of treatment. In general, a healthcare practitioner may prescribe the following medications, some of which are also commonly prescribed for those with lupus:

• Antimalarial hydroxychloroquine (Plaquenil), which has proven to be somewhat effective in treating Sjögren’s;
• Immunosuppressants such as methotrexate (Trexall);
• Arthritis medications such as disease-modifying anti-rheumatic drugs (DMARDs) to treat arthritis symptoms;
• Corticosteroids to manage inflammation;
• Eye drops such as cyclosporine (Restasis) or lifiitegrast (Xiidra) to treat chronic dry eye symptoms;
• Pilocarpine (Salagen) and cevimeline (Evoxac) to increase saliva production;
• Anti-fungal medications to treat yeast infections of the mouth, and
• Over-the-counter nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen or naproxen to treat joint pain and stiffness.

It is interesting to note that a review of the literature conducted in 2017 revealed that while hydroxychloroquine could be effective at lowering erythrocyte sedimentation rates – an indicator of inflammation – it is not necessarily that effective at treating dry mouth and dry eyes as compared to placebo. This review group was relatively small, however, and the researchers agreed that more aggressive studies need to be done.

Biologics such as cyclophosphamide (Cytoxan) and rituximab (Rituxan) may potentially be effective ways to treat Sjogren’s, but they are still under investigation. A 2015 study found that individuals who are treated with anti-B cell activating factor receptor (BAFF-R) belimumab may experience a reduction in Sjögren’s symptoms.  This could lead to more treatment options in the future.

Sometimes surgery may be recommended to treat dry eye.  In this procedure the ducts that drain tears from the eyes are actually sealed off with collagen or silicone plugs in order to conserve tears that are produced.

Some lifestyle changes may be implemented to help manage symptoms. These practices may include:

• using artificial tears or eye lubricants to alleviate the discomfort of dry eye;
• increasing the humidity in the home to bring moisture levels up;
• avoiding sitting in front of fans, HVAC vents or open windows which can exacerbate dryness;
• wearing goggles when outside to protect eyes;
• quitting smoking which can irritate and dry out the mouth.
• drinking water and avoiding caffeinated drinks, carbonated sodas and sports drink that are highly acidic and can cause dryness;
• chewing sugar-free gum or sucking on hard candies to stimulate saliva flow or using artificial saliva which may be more effective in some cases;
• using saline nasal spray to keep nasal passages clean and clear to ease breathing through the nose and avoid mouth breathing which can be incredibly drying;
• gently washing and moisturizing dry skin;
• staying on top of dental health and hygiene in order to prevent any serious mouth issues; and
• using vaginal lubricants to treat vaginal dryness.

Of course, practicing good self-care including stress-relief can also help individuals cope with any chronic illness such as Sjogren’s:

• engaging in activities that bring joy and happiness;
• practicing yoga, Tai chi or meditating in order to move gently and ease tension;
• reaching out to support systems or joining support groups that can provide a safe place to talk about coping with Sjogren’s;
• seeking the help of a therapist to find coping strategies and techniques;
• eating a well-balanced diet in order to maintain overall good health and a healthy weight; and
• getting plenty of quality rest and sleep and learning to set boundaries in order to preserve energy and stamina.

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Prognosis & Sjögren’s Disease Complications

To date, as with many of the autoimmune diseases, there is no known cure for Sjögren’s syndrome. As there is no cure, it is imperative that an individual is diagnosed and treated as soon as possible to prevent as much damage as possible and to reduce the possibility of complications.

Complications may still arise, however, and include:

• eye problems including permanent vision changes;
• lung issues such as scarring of the lungs and chronic lung infections;
• pregnancy complications that may affect the baby including skin rashes and heart problems;
• hypothyroidism;
• kidney issues such as kidney inflammation and the development of kidney stones;
• peripheral neuropathy;
• Irritable bowel syndrome (IBS)

In rare cases, an individual may be diagnosed with non-Hodgkin lymphoma. Though the number is still very small, the National Health Service states that individuals with Sjögren’s are five-times more at risk for developing cancer than those who do not have Sjögren’s. A 2016 study speculates that a “distinct genetic background” that is present at the time of a Sjögren’s diagnosis may pre-determine who will get non-Hodgkin lymphoma and who will not. For example, anti-Ro/SSA, anti-La/SSB and some rheumatoid factor (RF), which are present in individuals with Sjögren’s, are also present in individuals who have non-Hodgkin lymphoma alone.

In Conclusion

Like with SLE, Sjögren’s disease is a confusing and complex systemic condition that can present in a myriad of ways. Understanding the mechanisms of the disease, monitoring physical well-being and communicating new and concerning symptoms to a trusted healthcare practitioner is crucial for effective disease management.

 

References

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Author: Liz Heintz

Liz Heintz is a technical and creative writer who received her BA in Communications, Advocacy, and Relational Communications from Marylhurst University in Lake Oswego, Oregon. She most recently worked for several years in the healthcare industry. A native of San Francisco, California, Liz now calls the beautiful Pacific Northwest home.

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