Eyeworld

EW CORNEA 56 April 2018 by Vanessa Caceres EyeWorld Contributing Writer Then ophthalmologists will refer to rheumatology if necessary, Dr. Akpek said. Sometimes she has found it challenging for patients to get diagnosed even after they see a rheumatologist. "Rheumatologists do a vasculitis work-up, or they usually don't consider Sjögren's in the differential diagnosis of cor- nea ulcers or melt," she explained. Because of her background with Sjögren's patients, Dr. Akpek typi- cally orders a comprehensive blood work-up in patients with severe dry eye, sterile corneal ulcers, or intersti- tial keratitis. One test that has stimulated interest among practitioners on early Sjögren's diagnosis in the past 5 years has been the Sjö Diagnostic Test (Bausch + Lomb, Bridgewater, New Jersey). The test involves a simple finger prick for blood and analyzes for antinuclear antibody, Sjögren's-specific antibody-A (SS-A), SS-B antibody, and rheumatoid fac- tor, in addition to three proprietary biomarkers: anti-salivary protein-1, anti-carbonic anhydrase VI, and anti-parotid secretory protein. The three proprietary biomarkers are all found earlier in Sjögren's, said Anat Galor, MD, associate professor of clinical ophthalmology, Bascom Palmer Eye Institute, University of Miami. The test can be done in the office or at a lab, Dr. Galor said. "[The test] appears to increase the sensitivity and specificity of diagnosis in conjunction with tradi- tional testing," Dr. Kempf said. All insurance companies cover it except Aetna, he added. Both rheumatologists and oph- thalmologists have found the test useful. "I use it, and several other rheumatologists in my large practice use it and find it helpful to detect early Sjögren's, but data on the tests and rigorous academic testing is lacking," Dr. Kempf said. Because Sjögren's is often underdiagnosed or found late, Dr. Galor appreciates having a test like Sjö available, but whether early diagnosis with the test can lead to better outcomes isn't yet clear, she thinks. Once seen by a rheumatologist, a suspected Sjögren's patient will have a complete history and physi- cal and most likely also have an an- tinuclear antibody with titer and a full antibody profile to include SS-A Texas, San Antonio. With a diagno- sis, specialists also can monitor for malignancies such as lymphoma, which Sjögren's patients are at a higher risk of. Still, Dr. Kempf ac- knowledged the challenges of earlier diagnosis of Sjögren's. "Sjögren's patients tend to have a multitude of other less objective symptoms, like generalized pain and fatigue. Since there is no specific treatment for these symptoms, some rheumatol- ogists may take a less aggressive ap- proach in looking for early disease," he said. The ophthalmologist's role Dry eye is recognized by some prior surveys as the most bothersome symptom of Sjögren's. 3 Although ophthalmologists do not often diagnose Sjögren's, according to Dr. Akpek, patients should begin their journey to diagnosis and treatment at eye practices because of the prom- inence of dry eye symptoms. About 10% of patients with predominant- ly aqueous deficient dry eye have underlying Sjögren's, and at least half of them are unaware that they have it. 4 Aqueous deficient dry eye and sterile corneal ulcers are the two main findings that lead to a suspi- cion of Sjögren's, Dr. Akpek said. Other ocular complications may include corneal melting, corneal perforation, bacterial conjunctivitis, bacterial interstitial keratitis, and vision loss, Dr. Vivino reported. Ophthalmologists may not be involved with treating non-eye symptoms, but their suspicion should be higher if the patient also reports: • dry mouth • difficulty talking or swallowing • advanced dental cavities • joint pain • vaginal and skin dryness • overwhelming fatigue • gastrointestinal symptoms If Sjögren's is suspected, ophthalmologists should focus on drilling down dry eye symptoms through tests such as Schirmer's, ocular surface staining with fluores- cein/lissamine green, tear osmolari- ty, and tear breakup time. "Sjögren's patients may have aqueous tear de- ficiency from decreased tear produc- tion or excessive evaporation related to meibomian gland dysfunction/ blepharitis," Dr. Vivino reported. a patient will discuss tooth decay with their dentist, vaginal dryness with their gynecologist, and fatigue or joint pain with their primary care doctor," according to the Sjögren's Syndrome Foundation website. 2 "While each symptom alone wouldn't indicate an underlining autoimmune disease, when cou- pled together they would suggest Sjögren's." Earlier this year, the Sjögren's Syndrome Foundation announced that increased awareness about the disease and improved diagnostic methods have decreased time of disease onset to diagnosis from about 6 years to 2.8 years. 2 Lowering the time to diagnosis was part of a 5-year goal set by the foundation. Yet specialists familiar with Sjögren's want to continue to push for earlier diagnosis, to help patients improve their quality of life and get needed treatment. "With early diagnosis, you can prevent worsening of dry eye, and I tend to think that you can actually reverse or cure it," said Esen Akpek, MD, director, Ocular Surface Disease and Dry Eye Clinic, and associate director, Johns Hopkins Jerome L. Green Sjögren's Syndrome Center, Wilmer Eye Institute at Johns Hop- kins, Baltimore. Early diagnosis can help explain patients' symptoms and enable their physicians to monitor for organ-specific manifestations, such as interstitial lung disease, peripher- al neuropathy, or renal disease, said rheumatologist Kevin Kempf, MD, Rheumatology Associates of South Despite advances made in catching Sjögren's, many specialists are still out of the diagnosis loop E ven seasoned physicians, including ophthalmolo- gists, may underestimate the number of people who have Sjögren's syndrome— an autoimmune disease with rheu- matologic, ophthalmic, dental, and other symptoms. Sjögren's isn't rare; it's the sec- ond most common chronic autoim- mune disease within rheumatology, according to Frederick Vivino, MD, director, Penn Sjögren's Syndrome Center, Perelman School of Med- icine, University of Pennsylvania, Philadelphia, in a report published in Clinical Immunology. 1 As many as 4 million people in the U.S.—about 90% of them women—are living with Sjögren's, according to the Sjögren's Syndrome Foundation, Reston, Virginia. There is both primary Sjögren's, which is associated with aqueous deficient dry eye and dry mouth, and secondary Sjögren's, which has the same symptoms but takes place in conjunction with another auto- immune disease (e.g., rheumatoid arthritis or lupus). Because patients usually seek help from different specialists for various symptoms of Sjögren's, the typical time to diagnosis takes several years. "Diagnosing a patient quickly can be a challenge because often Diagnosing Sjögren's syndrome earlier

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