October 2018


Zeroing in on Sjögren’s dry eye

by Vanessa Caceres EyeWorld Contributing Writer

Typical triangular lissamine green staining of the bulbar conjunctiva within the interpalpebral fissure in a patient with Sjögren’s-related dry eye
Source: Esen Akpek, MD


Treatment can be challenging, requires multiple approaches

Treating severe dry eye in any patient can be a challenge. Treating severe dry eye in a patient who has Sjögren’s syndrome can be even more complicated than usual.
Dry eye is a common part of this disease. In fact, 97% of patients complained of dry eye in a report from the Sjögren’s Syndrome Foundation (SSF, Reston, Virginia).1 However, these patients may try numerous treatments on their own before they visit an ophthalmologist. At the same time, they may be seen by other specialists for their disease but not by eye professionals. Many take a long time to even be diagnosed with Sjögren’s—a journey that can take up 3 years on average, the SSF reported.2 This amount is actually a decrease from 6 years before, thanks to growing awareness about the disease, according to the SSF.
“By the time most of these patients get referred to me, they are often frustrated with the many different therapies they’ve tried with limited success,” said Ravi Patel, MD, Eye Associates of Central Texas, Round Rock. “I think part of that frustration is due to the fact that many other ailments are more easily categorized and treated, but due to the complexity of the ocular surface, this isn’t always the case.”
Although dry eye can be improved in many Sjögren’s patients, it cannot always be cured, said Esen Akpek, MD, Bendann Family Professor of Ophthalmology and Rheumatology, Wilmer Eye Institute at Johns Hopkins, Baltimore.
More effective dry eye treatment for Sjögren’s patients can help a large number of patients. As many as 4 million people in the U.S. have Sjögren’s, with the vast majority of them being women.3

Dry eye and quality of life

The severe dry eye that presents in Sjögren’s patients can require special care and attention, as it is often debilitating.
“Patients with chronic conditions can begin to see themselves differently and withdraw from certain activities that they previously enjoyed in hopes of reducing symptoms,” Dr. Patel said.
“Getting an early start with both the medical side and learning about preventive lifestyle changes puts you in a better position to deal with changes down the road,” said Rebecca Petris, founder, Dry Eye Company, Poulsbo, Washington. Ms. Petris provides resources and support to patients with dry eye and finds many with Sjögren’s who spiral into anxiety and depression as they manage information, specialists, treatments, and home remedies for all aspects of Sjögren’s, along with keeping up with their lives.
Depression and anxiety are commonly found among those with Sjögren’s, especially when dry eye is present. A study published in the American Journal of Ophthalmology focused on Sjögren’s patients found that dry eye complaints produced a 1.82-fold higher odds of depression compared with those who were symptom-free.4

Dry eye treatment plan for Sjögren’s

When a Sjögren’s patient presents with severe dry eye, there are several treatment moves that specialists will make.
Dr. Akpek likes to start with several treatments at once to jumpstart eye health. “I throw everything at the patient,” she said.
This includes over-the-counter preservative-free tears or serum tears (if the patient can afford the latter), an agent such as Restasis (cyclosporine, Allergan, Dublin, Ireland) up to four to eight times a day or Xiidra (lifitegrast, Shire Pharmaceuticals, Lexington, Massachusetts), and treatments for lid disease. She also sometimes adds a safe steroid but will have the patient’s IOP measured a few weeks after starting the steroid. She sees patients back at 6–8 weeks and usually notices improvement. However, if they need additional treatment, she will add tear duct closure or lid surgery.
Dr. Akpek also has success with the PROSE lens (BostonSight, Boston), although it does not prevent progress of dry eye disease in these patients. For some, she also considers secretagogues due to the concurrent presence of dry mouth.
This can be a lot for a patient to start at once, but compliance is surprisingly not an issue. “Sjögren’s patients are, for the most part, self-educated and very compliant,” she said.
Inna Ozerov, MD, Miami Eye Institute, Hollywood, Florida, also immediately starts Restasis at a dosing of three to four times a day. “The typical dry eye treatments need to be amped up in Sjögren’s patients in order to be effective,” she said. She will use tear duct plugs in all four tear ducts. When these are not enough, she is inclined to use amniotic membrane treatment.
Rupa Shah, MD, ReVision LASIK & Cataract Surgery, Columbus, Ohio, starts with punctal occlusion and adds Restasis or Xiidra as a second-line option. She sometimes adds ocular steroids for a short time period, depending on the inflammation present. Dr. Shah also frequently recommends preservative-free eye drops and warm compresses in the morning and at night, the latter of which help to improve meibomian gland dysfunction and decrease the evaporative component.
When patients have more severe signs or symptoms, Dr. Shah may use serum tears, intense pulsed light, a scleral contact lens, or an amniotic membrane placement.

Looking beyond the eye

Something that Dr. Patel likes to keep in mind when treating dry eye in Sjögren’s patients is confounding factors that may affect the condition. He gives the example of a patient who complained of dry eye that was particularly bad in the morning, with little relief. “I asked her about sleep apnea, and it turned out she had just started wearing a CPAP mask at night, but the mask was blowing air up her eyes,” he said. After the mask was refitted, her dry eye improved.
Dr. Patel questions what else may be happening if dry eye is unilateral, perhaps from HSV keratitis or an element of floppy eyelid syndrome in a patient who sleeps on one side of their face at night.
A review of environmental factors that can affect dry eye, including smoking, can help in some cases with symptoms, specialists said.
Because other systemic medications used by patients can worsen dry eye—including medications for depression and painkillers—Dr. Akpek will sometimes speak with other physicians to analyze if medication changes are possible. This does work sometimes, but Dr. Akpek said she is fortunate to work in a multidisciplinary setting devoted to Sjögren’s care.
When patients continually have trouble with treatments, it’s important to refer them to peers within rheumatology, as they may be able to update systemic medications that help with overall disease management, Dr. Ozerov said.
The need for concurrent care with other specialists is something that rheumatologist Scott Zashin, MD, volunteer clinical professor of medicine, University of Texas Southwestern Medical School, Dallas, thinks is critical for treatment. “I think it’s important that all my Sjögren’s patients are under the care of an ophthalmologist as they almost all have dry eyes,” he said. When his patients continue to have dry eye issues despite treatment, he will refer them to an ophthalmologist or corneal specialist with an interest in dry eye.

Looking for other treatments

Physicians are looking ahead to other options that may be useful in the future.
Although the TrueTear neurostimulator device (Allergan) is new to the market, Ms. Petris has seen positive comments about it in social media groups. TrueTear helps patients increase production of their own tears. She also has seen a growing interest in dry eye glasses to manage symptoms.
Dr. Shah is interested in both TrueTear and Dextenza (Ocular Therapeutix, Bedford, Massachusetts), a low-dose dexamethasone delivery system within a punctal plug. “This may prove promising for Sjögren’s patients who have severe inflammation,” she said.
There is interest in biologic therapy to treat Sjögren’s patients. For example, Dr. Zashin took part in a clinical trial with the rheumatoid arthritis drug Orencia (abatacept, Bristol-Myers Squibb, New York) to treat Sjögren’s. However, results are not yet published. Dr. Akpek would like to see more consideration of dry eye and Sjögren’s syndrome as pharmaceutical companies test or expand biologic indications and indications for other medications.


1. Sjögren’s Syndrome Foundation. Living with Sjögren’s 2016. www.sjogrens.org/home/about-sjogrens/livingwithsjogrenssurvey
2. Sjögren’s Syndrome Foundation. About Sjögren’s/Diagnosis. www.sjogrens.org/home/about-sjogrens/diagnosis
3. Vivino FB. Sjögren’s syndrome: Clinical aspects. Clin Immunol. 2017;182:48–54.
4. Gonzales JA, et al. How are ocular signs and symptoms of dry eye associated with depression in women with and without Sjögren syndrome? Am J Ophthalmol. 2018;191:42–48.

Editors’ note: The sources have no financial interests related to their comments.

Contact information

: esakpek@jhmi.edu
Ozerov: iozerov@gmail.com
Patel: rapatel5462@gmail.com
Petris: rebecca@dryeyecompany.com
Shah: rupas@revisioneyes.com
Zashin: szashin823@aol.com

Zeroing in on Sjögren’s dry eye Zeroing in on Sjögren’s dry eye
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