It’s no secret that autoimmune diseases can cause severe dry eye. One of the conditions that results in a more severe type of Dry Eye Syndrome is called Sjogren's Syndrome. In today’s video I’ll tell you all about it and how its treatment differs from that of Dry Eye caused by other factors. You’ve made it to Eye School with me, Dr. D. Welcome! Make yourself comfy, and get ready to learn about Sjogren's Syndrome.
So what is it?
Sjogrens is a chronic autoimmune disease. Autoimmune diseases occur when the body is attacking itself. Although Sjogrens is often listed as a dry eye/dry mouth disease, it is actually considered “Systemic” and therefore affects the whole body. Sjogrens is known for attacking the secretory glands, like the parotid that secretes saliva and the lacrimal that secretes the watery layer of your tears. That’s why Sjogrens is famous for causing dry eyes and a dry mouth.
Demographics
I had a mentor in school that told me you can often spot a Sjogren's patient because they’ll show up in your dry eye center with a huge bottle of water in tow and before the visit is over they’ll ask you where the water fountain is to refill it. It occurs most frequently in women, with 9/10 sjogren's patients being women. It’s estimated that as many as 4 million Americans have sjogrens with over half currently undiagnosed. It can occur in all age groups but the average age of diagnosis is 40 years old. It can often take over 2 years to receive a Sjogren's diagnosis.
Sjögren's Syndrome Symptoms, Tests, and Bloodwork
How it affects you . . .
In addition to dry eyes and a dry mouth, Sjogrens patients often have a general sense of fatigue as well as potentially some joint pain or digestive issues. This depends on the patient but is due to the fact that Sjogrens is a systemic autoimmune disease. The Sjogrens foundation also lists more systemic issues like dysfunction of the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas and even the nervous system.
Sjogren’s is fairly common in general and certainly very commonly seen in dry eye clinics like mine. However, often my patients have not yet been diagnosed with Sjogrens. Symptoms are often difficult to suss out because they overlap with other system wide conditions like other autoimmune diseases, effects of medications, or even menopause.
Tests and Bloodwork
As an eye doctor, I frequently run tests to diagnose Sjogrens. But, your dentist or rheumatologist is very likely to initiate testing as well. I often strongly suspect Sjogren’s in my
dry eye patients, especially if they have a history of dry mouth or cavities as well as in the case where there is a ton of inflammation and lack of tears WITHOUT extensive meibomian gland dysfunction, rosacea or blepharitis.
I use several blood tests for Sjogrens. SS-A (Ro) and SS-B (La) are marker antibodies for Sjogrens. I also ordered a panel called Sjo that measures 9 additional markers, and I have found this test to be more sensitive than simply testing Ro and La. Your doctor may also recommend a salivary gland biopsy where a small piece of tissue is taken from the lower lip. This test confirms lymphocytic infiltration of the minor salivary glands.
Early diagnosis of Sjögren’s syndrome (SS) in patients with clinically significant dry eye is relevant to prevent vision-threatening complications.[28] The Sjo test is an in-office diagnostic blood test. Finger prick blood sample is collected to saturate a specimen filter paper and sent to the lab.
The diagnostic testing panel includes four traditional SS antibodies (anti-SS-A/Ro, anti-SS-B/La, rheumatoid factor [RF], and antinuclear antibody [ANA]) along with three tissue
-specific autoantibodies (anti-SP-1, anti-PSP, and anti-CA-6). The 2012 American College of Rheumatology (ACR) classification criteria for SS suggested that a positive anti-SSA and/or positive anti-SSB or a combination of RF and ANA at a titer ≥ 1: 320 are needed to fulfill the positive serology criterion.[29] However, recently revised 2016 classification criteria included anti-SSA antibody as the only serological marker for SS. [30]
It is important to note that these tissue-specific antibodies are not included in any classification criteria for SS; therefore, they do not have a diagnostic value. Some of these antibodies were associated with more severe dry eye regardless of SS diagnosis. Based on current knowledge, a positive novel autoantibody may suggest either early stages of SS or another form of an autoimmune disease. [31][32][33][34]
Their utility is still under investigation. Strong suspicion of SS, even in the absence of the traditional or novel antibodies, should lead to a rheumatology referral as negative serology is not exclusive and further testing and evaluation, such as a lip biopsy, may still reveal the diagnosis of SS.
Sjögren's Syndrome Treatment and Case Study
Treatment
Since I’m an eye doctor and dry eye specialist, my focus (pun intended, obviously!) is on the treatment of Dry Eye related to Sjogrens. All of the typical Dry Eye treatments do apply for patients with Sjogren's Syndrome. Your doctor may recommend non-preserved artificial tears, modifying your diet, taking Omega 3 supplements, anti-inflammatories, or longer term inflammation control like Restasis, Cequa, or Xiidra.
Once inflammation on the ocular surface is controlled we will sometimes use punctal plugs to keep the tears on your eye longer. We can also use treatments like autologous serum drops (drops made from your own blood!!)to heal the ocular surface or amniotic membranes (dehydrated tissue from placenta!). When a patient has a diagnosis of Sjogrens with dry eye symptoms, it does change.
Case Study
Patient: 78y/o female with extensive history of DED. Eyes burn with restasis. Using a bag full of drops every day. Eyes feel terrible. This patient has concurrent meibomian gland dysfunction and other autoimmune diseases as well. When she came to see us, she had a ziploc bag full of medications and was taking Xiidra,
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