Patient Profiles in Sjögren's Syndrome

Patient Profiles in Sjögren's Syndrome

This activity is sponsored by Bausch + Lomb.

Detecting Sjögren’s Syndrome


Clifford L. Salinger, MD

Medical Director
VIP Laser Center
Palm Beach Gardens, FL

For many years, eye care professionals have been aware of the association of dry eye disease and Sjögren’s syndrome. The challenge has been to positively identify those individuals in whom Sjögren’s syndrome is a significant underlying factor contributing to their ocular condition. Multiple diagnostic modalities have failed in many cases to detect either the antibody via blood testing or the histologic signs in oral mucosal biopsy, because of lack of sensitivity or specificity. Studies have revealed that Sjögren’s syndrome significantly increases both morbidity and mortality because of associated systemic pathophysiology, with lymphoma being one of the more serious consequences.

The lack of awareness among our rheumatology and internal medicine colleagues became abundantly clear when a 54-year-old woman presented with complaints of chronic and progressive dry eye disease. The patient had significant and persistent ocular symptoms, with an Ocular Surface Disease Index score of 40, a Standard Patient Evaluation of Eye Dryness questionnaire maximum score of 28, and a Dry Eye Questionnaire 5 score of 18, despite long-term use of cyclosporine A (twice a day), well-positioned silicone punctal plugs in both lower eyelids, frequent use of preservative-free tears (approximately 6 times a day), and daily omega-3 supplements. She had also been diagnosed with “burning mouth syndrome” and “chronic fatigue syndrome,” as defined by her primary care physician, and “post-menopausal vaginal atrophy with vaginal dryness related to possible Sjögren’s syndrome,” as noted by her rheumatologist. Further questioning revealed additional symptoms consistent with Sjögren’s syndrome including intermittent myalgia, lower extremity achiness and joint pains and an “abnormal weight loss” of about 10 lbs in the past couple of months, coincident with increased symptomatology. Previous evaluations revealed a “negative antibody profile.”

"We now can diagnose Sjögren’s syndrome in patients on average, almost 5 years earlier than with traditional antibody profile and tissue biopsy."

With repeated encouragement, the patient consented to have blood drawn for the Sjö® diagnostic test (Bausch + Lomb), which includes the Early Sjögren’s Syndrome Profile. The test identifies the presence or absence of unique biomarkers consistent with Sjögren’s syndrome separate from the standard or traditional ones that are routinely tested. Results revealed a slightly elevated carbonic anhydrase 6, immunoglobulin G (IgG) value of 22.1 EU/mL (normal value ‹20.0), and immunoglobulin M (IgM) value of 22.5 EU/mL (normal value ‹20.0). The rheumatologist’s conclusion was “possible seronegative Sjögren’s syndrome” based on her global symptomatology, despite the finding of a “mildly positive carbonic anhydrase Ab, which may be positive in some patients years before other antibodies but is still not part of the diagnostic criteria.” The rheumatologist also noted that “in light of negative (traditional) antibody profile,” it was recommended to perform an additional work-up to rule out Sjögren’s syndrome mimics, including hepatitis C and lymphoma. This included a lip (salivary gland) biopsy, which failed to obtain sufficient tissue sample for evaluation, as reported by the patient, and she refuses to have the biopsy repeated.

The Sjö test, with four proprietary biomarkers — and, therefore, inherently increased sensitivity — has greatly improved our diagnostic capability to identify these individuals. With this test, we can diagnose Sjögren’s syndrome in patients on average, almost 5 years earlier than with traditional antibody profile and tissue biopsy. These patients can thus be treated much earlier during their disease, which reduces both the consequent morbidity and mortality of their condition. Eye care professionals are in a unique position to diagnose Sjögren’s syndrome in these individuals and save them years of misery because dry eye disease is often one of the earliest manifestations of their condition. I believe that it is our collective responsibility to educate our medical colleagues regarding the critical importance of actively identifying these individuals, defining the presence of their disease and treating them earlier and more aggressively to minimize the potential for long-term sequelae.

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