Sjögren’s syndrome screening may have major impact on dry eye

Take home

A new test for the diagnosis of Sjögren’s syndrome may change ophthalmic practices’ approach to the management of dry eye.

Dr. Ayers

By Brandon D. Ayres, MD, Special to Ophthalmology Times

In the past, Sjögren’s syndrome has been challenging to diagnose, with limited tools and invasive procedures. Traditional biomarkers associated with detecting Sjögren’s syndrome are less likely to detect the disease at an early stage.¹

Often, patients who have been experiencing dry eye symptoms for years-even decades-are referred without ever having been tested for Sjögren’s syndrome. This is not surprising, as there is an average delay of 4.7 years for patients to receive an accurate diagnosis once they begin seeking treatment for their symptoms.²

Unfortunately, a delay in diagnosis can lead to systemic manifestations that can be debilitating, such as musculoskeletal pain, kidney dysfunction, and risk of lymphoma, to name a few. Eye-care professionals (ECPs) are in a unique position to detect Sjögren’s syndrome early, as many see dry eye patients on a regular basis, and one of the early hallmark symptoms of this disease is dry eye.

A new diagnostic test for Sjögren’s syndrome is changing the way dry eye is managed, because ophthalmologists are able to rule in or rule out the disease as an underlying cause of dry eye. By intervening early, Sjögren’s syndrome can be detected and ophthalmologists can ensure patients are referred to other specialists for appropriate management, which lessens the risk of complications and ultimately improves their quality of life.

The test

Considering as many as one in 10 patients with dry eye may also have Sjögren’s syndrome, all patients who present to our practice with ocular surface disease now are screened with an advanced diagnostic test (Sjö, Nicox) for the early detection of Sjögren’s syndrome for patients with dry eye.³

By testing patients early, the goal is to catch Sjögren’s syndrome before patients are in the late stages of the disease and gland degradation has taken effect.

The test can be administered in the office by a technician. The provided lancet is used to prick the patient’s finger and to collect blood drops on the included specimen collection card. After enough blood has been collected to perform the test adequately, the collection card is allowed to dry completely.

A request form for the test is filled out with the patient’s personal information (including medical insurance) as well as the practice’s information, before the packet is sent in a preaddressed delivery service envelope (FedEx) to the testing laboratory. Within a week, a report is received via e-mail or fax with the test results.

The test has a significant advantage over previous screening approaches for Sjögren’s syndrome in which we tested for traditional biomarkers that were only expressed in a low percentage of patients and that typically appeared later in the disease process. In addition to testing for the presence of the traditional biomarkers, the new test also screens for three novel, proprietary biomarkers-salivary protein-1 (SP-1), carbonic anhydrase (CA-6), and parotid secretory protein (PSP)-that have a higher sensitivity and specificity because they are gland-specific to the lacrimal and salivary glands.

Results and patient response

Patients are responding positively to the new screening protocol for Sjögren’s syndrome. They are excited because, often patients with dry eye are looking for an answer. They want to know why they have dry eye and why previous treatments may have proven ineffective.

As a result, I have never had a patient refuse the test. If a test returns positive, patients are given a reason for their dry eye symptoms and they are glad to have an answer that can be pinpointed and treated. Even if a test indicates that a patient is unlikely to have Sjögren’s syndrome, at least we are able to rule out the disease as an underlying cause of dry eye for the time being. Patients who test positive for Sjögren’s syndrome receive a letter for their primary-care physician recommending referral to a rheumatologist for further treatment.

Clinical case

A 62-year-old woman was referred by a corneal specialist for management of severe dry eye. Her primary complaints on the initial visit were constant irritation in both eyes as well as reduced vision. She also mentioned that she was experiencing dry mouth and needed to drink water all day.

The patient had already been treated with preservative free artificial tears, topical cyclosporine, punctal plugs, doxycycline, and omega-3 fatty acids. Upon examination, her visual acuity was reduced to 20/30 and 20/60. A slit lamp examination revealed moderate blepharitis with a low tear volume. Her interpalpebral conjunctiva was noted to have extensive lissamine green staining and both corneas had fluorescein staining in the lower third. The remainder of the anterior segment was normal with the exception of mild cataracts.

The patient was referred to rheumatology for a SjÓ§gren's work-up, and autologous serum drops were added to her topical regimen. Screening in rheumatology was negative and no systemic therapy was instituted. The patient’s condition responded well to the addition of autologous serum and she returned every 3 months for additional serum drops.

On a follow-up examination, the Sjögren’s syndrome screening test was performed, which showed several positive markers indicating high suspicion for the syndrome. The patient was returned to her rheumatologist for further investigation and institution of systemic treatment.

The new screening protocol is a point of differentiation. Although our practice is known for being aggressive with diagnosing ocular surface disease in patients, the test should be in the office of every ophthalmologist.

It is hoped that, at some point, screening for Sjögren’s syndrome is not a point of differentiation, because one of ophthalmologists’ responsibilities is to screen patients for serious diseases. SjÓ§gren’s syndrome is not only an eye health concern, but also a public health concern. It affects so many other systems in the body, and it is something that all ECPs, no matter the size of their practice, can provide.

References

1.     Shen L, Suresh L, Lindemann M, et al. Novel autoantibodies in Sjögren’s syndrome. Clin Immunol. 2012;145:251-255.

2.     Sjögren’s Syndrome Foundation. Sjögren’s Syndrome Foundation. 2001. Available at http://www.sjogrens.org. Accessed April 15, 2014.

3.     Liew M, Zhang M, Kim E, et al. Prevalence and predictors of Sjögren’s syndrome in a prospective cohort of patients with aqueous-deficient dry eye. Br J Ophthalmol. 2012;96:1498-1503.

Brandon D. Ayres, MD, is a surgeon in the Cornea Service, Wills Eye Hospital, Philadelphia. Readers may contact Dr. Ayres at 484/434-2700 or bayres@willseye.org. He did not indicate any proprietary interest in the subject matter. The test is marketed in the United States and is not available in Europe and the rest of the world.