Primary Sjögren’s syndrome is an autoimmune disorder that has an estimated incidence of 4 per 1,000 patients, affecting all ethnic and racial groups (Both et al, 2017; www.sjogrens.org ). Lymphocytic infiltration of the secretory glands results in sicca syndrome, which causes dryness of the eyes and mouth. Patients who have Sjögren’s syndrome may also suffer from chronic pain, fatigue, and neuropathies (www.sjogrens.org ). Primary Sjögren’s syndrome caries a five-fold higher relative risk of lymphoma, an extraglandular manifestation with the highest mortality (Both et al, 2017).

Sjögren’s syndrome can occur in patients who have other autoimmune disorders such as rheumatoid arthritis or lupus. An estimated 20% of rheumatoid arthritis patients has secondary Sjögren’s syndrome (Both et al, 2017).

Diagnosis

Sjögren’s syndrome, as with other autoimmune disorders, can be difficult to diagnose and can take years to identify. The non-specificity and range of severity of its symptoms contribute to this delay. Diagnosis has been based upon the American-European Consensus Group (AECG) classification criteria, which include subjective ocular dryness, subjective oral dryness, objective measure of dryness from Schirmer’s test or corneal stain, salivary biopsy, salivary scintigraphy, and presence of autoantibodies (Both et al, 2017).

Recently, the American College of Rheumatology has published a revised classification system that removes the subjective components (Franceschini et al, 2017), but the AECG classification is still commonly used in clinical practice (Both et al, 2017).

Management

Patients who have Sjögren’s syndrome are best managed by a team of clinicians, which typically includes a rheumatologist, a dentist, and an eyecare practitioner. In addition to taking disease-modifying antirheumatic drugs (DMARDs) such as hydroxychloroquine, patients are often prescribed muscarinic receptor agonist to stimulate salivary function as well as topical ophthalmic therapy for management of dry eye symptoms.

Cyclosporine (0.05%), lifitegrast, autologous serum, and over-the-counter artificial tears are often prescribed for dry eye treatment. Bandage soft contact lenses or scleral contact lenses are an option for many of these patients who have severe disabling dry eye symptoms that aren’t successfully managed with topical ophthalmic therapy. Even for mild symptoms, scleral lenses in particular may allow patients who have Sjögren’s syndrome to wear contact lenses to concurrently correct refractive error and manage ocular surface dryness.

Topically prescribed ophthalmic medical drop frequency will often need to be adjusted during scleral lens wear. Prescription drops should be applied before lens wear or after lens removal; however, patients can continue nonprescription artificial tears during scleral lens wear. Make sure to communicate possible changes in therapy with any other eye specialists who are managing the patient.

Fitting Considerations

Mini-scleral lenses (15.5mm to 17.5mm) or full scleral lenses (18mm and larger) can be utilized depending on a patient’s ocular anatomy. A significant factor is to ensure that the fitted scleral lens has complete corneal clearance. Choose mini-scleral lenses for patients who have smaller apertures or less severe dryness. Prescribe full scleral lenses for patients who have large corneas or severe dryness in which more surface coverage of the anterior segment can be beneficial.

Conclusions

Managing dry eye with scleral contact lenses is often an over-looked option for Sjögren’s syndrome patients. Educating practitioners at local tertiary care centers is a way to help more patients who have Sjögren’s find relief with scleral contact lenses. CLS

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