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SjÃ¶gren’s syndrome (SS) was named after Henrik SjÃ¶gren, MD, a Swedish ophthalmologist.
Coincidentally, in Swedish “sjÃ¶” translates to “lake,” and SS is related to a reduced tear lake. In 1933, Dr. SjÃ¶gren was the first to identify three classical symptoms of keratoconjunctivitis: sicca, xerostomia, and polyarthritis.1
SS currently affects 4 million Americans, with more than half of patients going undiagnosed. The problem with diagnosing SS is that it may present in different ways and manifest as one symptom or multiple symptoms.2
Related: Understanding SjÃ¶gren’s syndrome
Some of these symptoms include dry eyes, dry mouth, fatigue, joint ache, and depression. Patients may be seeking different specialties for each symptom without specialists piecing together that the constellation of symptoms are all from SS. In 2012, the timeline from onset of SS to an accurate diagnosis took six years.
However, with the efforts of the SjÃ¶gren’s Syndrome Foundation to increase awareness, the time to obtain an accurate diagnosis was reduced to 2.8 years just five years after the awareness campaign began. Earlier diagnosis means patients obtain treatment faster.3
Here is a review of recent important updates about SS and dry eye disease (DED).
Related: Blog: Don't leave dry eye disease patients high and dry
1. New guidelines for diagnosing primary SjÃ¶gren’s syndrome
When was the last time you used the Schirmer test? An OD may want to have it handy and available at her office if she wants to follow the guidelines outlined in the “2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary SjÃ¶gren’s Syndrome.”4
The criteria require both subjective and objective components to meet the new guidelines.
First, determine if a patient has at least one symptom of ocular or oral dryness defined as a positive response to at least one of the following questions:
1. Have you had daily, persistent, and troublesome dry eyes for more than three months?
2. Do you have a recurrent sensation of sand or gravel in the eyes?
3. Do you use tear substitutes more than three times a day?
4. Have you had a daily feeling of dry mouth for more than three months?
5. Do you frequently drink liquids to aid in swallowing dry food?
If a patient meets at least one of the positive responses to at least one of the symptoms in the subjective component, then the objective criteria is applied.
Individuals who have a total score of ≥4 for objective criteria and have at least one positive response to the subjective criteria meet the criteria for SS based on the 2016 guidelines. Labial salivary gland biopsy and anti-SSA/Ro are heavily weighted with 3 points each, while the other factors are all 1 point each.
Thus, to reach a score of 4 or greater, the labial salivary bland biopsy or the anti-SSA/Ro must be positive.4
2. Potential new dry eye medication for SjÃ¶gren’s
ODs are aware of the advanced complications from DED, especially when patients have SS. While it takes months to manage and control DED, SS patients suffer from severe discomfort and significant visual compromise.
There may be a new medication on the horizon that may help SS patients with their dry eyes.
Related: Blog: Why dry eye?
Although the clinical trial was conducted to establish safety and efficacy, preliminary results show promise for treating DED in a relatively short amount of time.
A clinical trial published this past spring used preservative-free deoxyribonuclease (DNase) 0.1% eye drops to reduce the signs and symptoms of DED.5
The medication was administered four times a day for eight weeks in 47 patients with severe tear-deficient DED. The study included 53 percent SS patients, 30 percent non-SS patients, and 17 percent ocular graft vs. host disease patients.
This was the first clinical trial using DNase on human eyes. In just eight weeks, the results showed significant and clinically meaningful reduction in corneal staining compared to placebo. The most frequent adverse event reported in 20 percent of patients was temporary burning upon instillation.
ODs recognize that ocular surface inflammation plays a key role in the pathogenesis of DED. Patients with severe DED have numerous neutrophils on the ocular surface that release nuclear chromatin complex, a type of extracellular DNA (eDNA) web that forms neutrophil extracellular traps (NETs).5,6 Related: Blog: Examine evaporative dry eye disease exposure in your patients
In DED, increased osmolarity and/or tear deficiency increases NETs exponentially on the ocular surface, which correlates with increased signs and symptoms of DED.5,6
DNase is a major extracellular endonuclease that acts as a “waste-management enzyme” to selectively cleave DNA that leaks into the extracellular space when the cells die.
DNase is currently available as Pumozyme (dornase alfa, Genetech) and is approved as a daily-inhaled solution for patients with cystic fibrosis.5
Based on this recent study on DED, DNase shows promise in treating moderate to severe DED, and the eyecare community looks forward to seeing additional studies.
Related: Identify SjÃ¶gren's patients with SjÃ¶ test
3. SjÃ¶ test: Is it still relevant today?
The SjÃ¶ Diagnostic Test was initially launched in 2013 by Nicox, which sold to Bausch + Lomb.
This lab test is a diagnostic panel that includes detection of classic biomarkers for SS, lupus, and rheumatoid arthritis as well as novel early biomarkers for early SS.
Consider using this interactive tool from Immco to help interpret SjÃ¶ test results.
I have been using the SjÃ¶ test since it first launched, and I believe it should be included in an OD’s dry eye toolbox. Research has shown that 10 percent of dry eye patients may have SS.7
SS patients may also have a higher risk of developing non-Hodgkin’s lymphoma, a cancer that may be treatable if found early. By using the SjÃ¶ test for dry eye patients, an OD may be the one doctor who could make the difference in saving a patient’s life.
Read more dry eye awareness content here
1. Firestein GS, Budd RC, Harris ED, McInnes IB, Ruddy S, Sergent JS, editors. Kelley and Firestein’s Textbook of Rheumatology. 8th edition. Philadelphia: W.B. Saunders, 2006. Print.
2. SjÃ¶gren’s Syndrome Foundation. About SjÃ¶gren’s. Available at: https://www.sjogrens.org/home/about-sjogrens. Accessed 7/22/19.
3. SjÃ¶gren’s Syndrome Foundation. SjÃ¶gren’s Syndrome Foundation Accomplishes 5-Year Breakthrough Goal. Available at: https://sjogrens.org/home/about-the-foundation/breakthrough-goal-/5yearupdate. Accessed 7/22/19.
4. Shiboski CH, Shiboski SC, Seror, R, Criswell, LA, Labetoulle, M, Lietman TM, Rasmussen, A, Scofield H, Vitali C, Bowman SJ, Mariette X, International SjÃ¶gren's Syndrome Criteria Working Group. 2016 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Primary SjÃ¶gren's Syndrome: A Consensus and Data-Driven Methodology Involving Three International Patient Cohorts. Arthritis Rheumatol. 2017 Jan;69(1):35-45.
5. Akpek EH, Bunya VY Saldanha IJ. SjÃ¶gren’s Syndrome: More Than Just Dry Eye. Cornea 2019 May;38(5):658-661. doi: 10.1097/ICO.0000000000001865. 6. Mun C, Gulati S, Tibrewal S, Chen YF, An S, Surenkhuu B, Raju I, Buwick M, Ahn A, Kwon JE, Atassi N, Pradeep A, Rondelli D, Jain S. A Phase I/II Placebo-Controlled Randomized Pilot Clinical Trial of Recombinant Deoxyribonuclease (DNase) Eye Drops Use in Patients With Dry Eye Disease. Trans Vis Sc. Tech. 2019;8(3):10. doi: 10.1167/tvst.8.3.10.
6. Akpek EH, Bunya VY Saldanha IJ. SjÃ¶gren’s Syndrome: More Than Just Dry Eye. Cornea. 2019 May;38(5):658-661. doi: 10.1097/ICO.0000000000001865.
7. Mun C, Gulati S, Tibrewal S, Chen YF, An S, Surenkhuu B, Raju I, Buwick M, Ahn A, Kwon JE, Atassi N, Pradeep A, Rondelli D, Jain S. A Phase I/II Placebo-Controlled Randomized Pilot Clinical Trial of Recombinant Deoxyribonuclease (DNase) Eye Drops Use in Patients With Dry Eye Disease. Trans Vis Sc. Tech. 2019;8(3):10. doi: 10.1167/tvst.8.3.10.