Sjogren's Disease

Sjogren’s Disease (SD) (pronounced SHOW-grins) is a chronic, progressive, multi-system autoimmune disease characterized by lymphocytic infiltration of the exocrine glands.1,2,3 SD is the most common systemic autoimmune rheumatic disease, affecting over 3 million individuals in the United States.4,5 However, SD is often unrecognized and underdiagnosed due to the varying presentations of ocular and systemic manifestations and patient signs and symptoms, most commonly dry eye and/or dry mouth.2,3,5 It has been reported that there is an increased prevalence of SD in women in the second to third decade or in the fourth to fifth decade.6,7,8,9,10

Similar to other autoimmune disorders, when you have Sjögren's your body’s white blood cells attack and damage your own body. In this case, the disease targets the moisture-secreting glands of the eyes and mouth, resulting in decreased production of tears and saliva.

This being said, Sjögren's can damage other parts of your body as well, such as your joints, kidneys, liver, lungs, nerves, skin and thyroid. Furthermore, Sjögren's disease does not always occur alone—it often accompanies other immune disorders, such as rheumatoid arthritis, lupus, celiac disease or scleroderma.

Men and women of any age or race can develop Sjögren's disease, but it is most common among Caucasian women who usually start recognizing symptoms between ages 40 and 60.

In the universe of autoimmune disorders, Sjögren's is one of the most common. In fact, four million Americans have the disease, and as many as three million may be unaware they have the condition.

Since dry eye is one of the primary symptoms of Sjögren's, it is important to make sure your eyes are lubricated if you have this disease, since failure to do so can lead to scarring and infection as well as general discomfort.

What Causes Sjögren's Disease?

Autoimmune disease, in general, is not completely understood. In healthy people, the body is programmed to attack foreign substances; but in people with autoimmune diseases like Sjögren's, the body wages attack on itself. This abnormal response has confounded doctors for years, inspiring a wealth of research on the topic. Much of the research focuses on the heritability of Sjögren's.

Many reports have illustrated that certain genes can put you at higher risk of developing Sjögren's; however, it is not clear why some people develop the disease while others do not. Many clinicians believe that a virus or other infection is needed to set Sjögren's into motion. Indeed, even other autoimmune diseases seem to trigger Sjögren's since the condition so frequently presents alongside other autoimmune diseases.

How Is Sjögren's Disease Diagnosed?

Sjögren's can be hard to recognize since symptoms mimic those caused by several other diseases. Most often, complaints of dry eyes or a dry mouth are what precipitate a medical investigation, particularly if there are other red flags, such as joint pain, fatigue or a rash.

If your doctor suspects Sjögren's Disease, certain tests may be performed:

• Eye tests. Your eye doctor may perform a Schirmer's test, in which a small strip of paper is inserted under your lower eyelid to measure the amount of tears you produce. The doctor may also put a bit of dye in your eye to see how quickly your tears evaporate and to determine if dryness is causing any damage to your ocular structures.

Indeed, a number of tools that are commonly utilized for the diagnosis of dry eye can be used to help diagnose Sjögren's Disease. These include patient symptom questionnaires, tear film osmolarity, corneal and conjunctival staining with fluorescein, rose bengal and lissamine green, tear break up time, Shirmer’s testing, non-invasive measurements of tear film quality and quantity, etc. However, there is a lack of consistent dry eye testing among clinics and there is currently no gold standard for the examination or documentation of dry eye disease in SD patients.

• Saliva tests. If you are suspected of having Sjögren's syndrome, the amount and quality of your saliva may be measured. In addition, doctors can perform an imaging test to check how well your salivary glands function. A lip biopsy also may be examined to detect clusters of inflammatory cells that are commonly seen in people who have Sjögren's syndrome.

• Blood tests. Since certain antibodies in the blood are commonly found in people with Sjögren's, blood tests are frequently used to reveal whether or not you have the disease. These tests have become more sensitive in recent years since doctors are now able to test for additional Sjögren's biomarkers.

Unfortunately, despite the abundance of available tests, many cases of Sjögren's go unreported. This may be due to the fact that dry eye itself is so common. However, it is estimated that one in ten dry eye patients also have Sjögren's and it takes an average of 3.5 years from the onset of symptoms to get an accurate diagnosis.

Medical Treatments for Sjögren's Disease

There are several home remedies for Sjögren's including over-the-counter artificial tears or ointments, increased water intake, and use of a home humidifier. When these fail to do the trick, prescription medications may be needed.

The medications that are most commonly used to treat Sjögren's are those that aim to increase production of saliva, suppress the immune system, or address specific complications such as inflammation or pain in the joints or on the ocular surface.

Another approach to treating the dry eye that accompanies Sjögren's is called punctal occlusion. This procedure is performed in your eye doctor’s office. Collagen or silicone plugs are inserted into your tear ducts to prevent the excess drainage of tears from your eyes. Since the effects of punctual occlusion are only temporary, in some cases, your doctor may suggest permanently sealing your tear ducts using a laser.

Scleral Lenses for Sjögren's Disease

Scleral lenses are a relatively new type of treatment for the ocular dryness associated with Sjögren's Disease.  A scleral lens is a large-diameter contact lens that vaults across the entire corneal surface and rests on the white part of the eye, known as the sclera. Liquid fills the space between your eye and the back surface of the scleral lens. This liquid bandage protects the cornea from the ongoing mechanical shearing effect of the eyelids that occurs when you blink. It also continuously bathes your sensitive corneal tissue.

If you have Sjögren's and also require vision correction, the scleral lens is additionally helpful because other contact lens options may be limited by the compromised state of your ocular surface.  As a result, you will most likely be unable to tolerate conventional soft lenses. Fortunately, scleral lens designs can overcome these challenges – these lenses work especially well for vision correction because the fluid layer helps smooth out defects caused by dry eyes, providing a more uniform refractive surface.

Research on Sjögren's Disease has evolved dramatically in recent years. If you’ve been diagnosed with this disease or have dry eyes and suspect it could be Sjögren's, visit your eye doctor soon to learn about new strategies that might work for you.

 


References

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[3] Beckman KA, Luchs J, Milner MS. Making the diagnosis of Sjögren’s syndrome in patients with dry eye. Clin Ophthalmol. 2016;20 :43-53.

[4] Helmick CG, Felson DT, Lawrence RC, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. 2008;28 :15-25.

[5] Caffery B, Simpson T, Wang, S, Bailey D, et al. Factor Analysis of the Clinical Characteristics of Primary Sjogren Syndrome. Optom and Vis Sci. Oct2010;87(10):742-750.

[6] Fox RI. Sjögren’s syndrome. Lancet. 2005;366:321-31.

[7] Helmick CG, FElson DT, Lawrence RC, et al. National Arthritis Data Workgroup. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Arthritis Rheum. 2008;58:15-25.

[8] Manthorpe R, Asmussen K, Oxholm P. Primary Sjögren’s syndrome: diagnostic criteria, clinical features, and disease activity. J Rheumatol Suppl. 1997;50:8-11.

[9] Patel R, Shahane A. The epidemiology of Sjögren’s syndrome. Clin Epidemiol. 2014;6:247-255.

[10] Bjerrum K, Prause JU. Primary Sjögren’s syndrome: a subjective description of the disease. Clin Exp Rheumatol. 1990;8:283-288.