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Include Sjögren’s in your differential diagnosis of dry eye
Differentiating between this syndrome and dry eye is critical in determining a management plan.
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Optometrists will see many patients with dry eye in their practices. However, when these patients also present with dry mouth, two conditions by which Sjögren’s syndrome is manifested, the clinician should investigate further.
Paul M. Karpecki, OD, said if a patient comes for their eye exam with a bottle of water, or if a patient positively identifies dry mouth symptoms, that would make him suspect Sjögren’s syndrome.
“Another thing would be if the patient mentions they have difficulty crying at sad movies, or even funerals. If they can’t cry at times when crying commonly occurs, then I might think Sjögren’s,” he said in an interview with Primary Care Optometry News.
Other indicators include the ulnar shift in a patient’s hands indicative of rheumatoid arthritis or any systemic autoimmune disease, Karpecki said.
Diagnosing Sjögren’s
According to the Sjögren’s Syndrome Foundation (SSF), early diagnosis and treatment are important for preventing complications, but it typically takes more than 5 years after the onset of symptoms to make a diagnosis.
Although Sjögren’s occurs in all age groups, including children, nine out of 10 patients are female, a SSF press release said, and the average age of onset is late 40s.
All instances of Sjögren’s are systemic, the release said. It can cause a patient’s kidneys, gastrointestinal system, lungs, liver, pancreas and nervous system to become compromised and possibly even shut down. Symptoms typically include dry mouth, dry eye, vaginal dryness, fatigue and joint pain.
“In many cases, however, ocular symptoms and signs are the first and sometimes only evidence of the disorder,” Art Epstein, OD, told PCON. “Hence, one of the challenges of the disorder for eye care providers is establishing a proper diagnosis when a potential Sjögren’s patient presents with dry eye. In other words, does the patient have dry eye, or is their dry eye a manifestation of Sjögren’s syndrome?”
The ocular signs of Sjögren’s include significant corneal and conjunctival staining, filamentary keratitis, a low non-anesthetized Schirmer’s test score and high osmolarity readings (above 340 in either eye), all combined with xerostomia, or dry mouth. This makes the correlation to systemic disease strong, according to Karpecki.
New diagnostic test
Presently, the only reliable way to test for Sjögren’s is a salivary gland or lip biopsy; however, a new test in development could provide clinicians a positive diagnosis much earlier in its progression.
“Right now, we typically only diagnose it late and in the most severe patients,” Karpecki said. “By that point it may be too late to fully reverse the effects on the cornea, lacrimal glands and ocular surface.
Paul M. Karpecki
“Furthermore, not only may patients with Sjögren’s syndrome require a secondary autoimmune systemic disease treatment due to the risks in conditions such as lupus, but they are also 46 times more likely to have B-cell non-Hodgkin’s lymphoma,” he said. “This is a treatable form of cancer if caught early, and Sjögren’s patients should be monitored for this yearly. Thus, the benefits of an earlier Sjögren’s diagnosis for these patients are extensive.”
The test, currently being developed by Nicox and Immco, utilizes three new biomarkers for Sjögren’s that have recently been discovered: salivary gland protein-1 (SP-1), carbonic anhydrase-6 (CA-6) and parotid secretory protein (PSP), according to the Nicox website.
“The traditional tests use ANA, Ro, La and RF antibodies, which have significant limitations of sensitivity and/or specificity and are associated with later-stage disease,” Karpecki said. “During studies (Shen et al.), these novel antibodies were found in 45% of patients meeting the criteria for Sjögren’s syndrome who lacked the traditional antibodies for Ro or La. In patients diagnosed with idiopathic xerostomia and xerophthalmia for less than 2 years, 76% had antibodies to SP-1 and/or CA-6, while only 31% had antibodies to Ro or La.”
The diagnostic test was approved earlier this year and is available to eye care practitioners in Canada, Puerto Rico and Mexico, according to the Nicox website. Commercial launch of the test in the U.S. is expected some time in the second half of 2013, the site said.
Managing Sjögren’s
“Optometry can manage Sjögren’s patients very effectively,” Karpecki said. “I currently have more than 160 patients in my clinic with a positive diagnosis.”
Epstein stressed the importance of aggressive treatment, even in the absence of ocular symptoms.
“I’ve found that you can easily get behind the therapeutic [power] curve, especially with patients who have a significant aqueous deficiency or inflammatory component,” he said. “I am especially observant of meibomian gland function because this is one area where we can usually intervene successfully, especially early on, and improve or maintain tear quality and quantity.”
Karpecki said that he has placed nearly half of his Sjögren’s patients on oral secretagogues.
“I particularly like Evoxac (cevimeline, Daiichi), 30 mg, two or three times daily, but it can be costly, so I’ll use Salagen (pilocarpine HCl, Patheon) 10 mg to 15 mg per day,” he said. “However, Salagen has many more side effects, in my experience, such as back or scalp sweats. I primarily make this decision based on the xerostomia more than the dry eye, but medications like Evoxac seem to help with the dry eye as well.”
Epstein supported the team approach.
“It is good for the patient and it increases our exposure and interaction with the medical community,” he said. “In cases where Sjögren’s patients have only ocular findings, most optometrists can successfully manage the patient on their own. But when a dry eye patient begins to show other signs or symptoms consistent with Sjögren’s syndrome, it’s wise to refer them to their primary care physician, rheumatologist or dentist (if the symptoms are primarily oral).”
A specialist should also be involved if signs are not being controlled, Karpecki said, if the condition is progressing under treatment or if a high risk of corneal scarring develops, such as corneal neovascularization, corneal thinning or a significant loss in vision, he said.
“It is imperative to get patients to a rheumatologist, or an internist with a specialty in this area, early for confirmation of the diagnosis and systemic therapy,” Karpecki said.
Art Epstein
“Managing a Sjögren’s patient is complex,” Epstein said. “In addition to the well known and classically described aqueous deficiency associated with Sjögren’s, many patients have concurrent evaporative dry eye secondary to meibomian gland dysfunction (MGD). I believe it’s impossible to fully manage most of these patients without attention to both aqueous and evaporative components.
“Therefore, patients should be treated holistically, targeting all aspects of their dry eye disease including using lipid-based tear supplements and, when appropriate, MGD treatment, in addition to collaborating with the patient’s primary care physician for their systemic management,” Epstein concluded. – by Daniel R. Morgan
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Disclosures: Epstein and Karpecki are consultants to Nicox.