ABSTRACT
Sjögren Syndrome (SS) is one of the most frequent systemic autoimmune disorders, mainly involving the eye and mouth due to inflammation of lacrimal and salivary glands. Exocrine glands affected with a typical focal lymphocytic infiltration potentially lead to dry eyes and dry mouth. In addition to the known pathogenic mechanism of SS through autoimmunity, corneal neuropathy, as a peripheral neuropathy which is a relatively frequent extraglandular systemic manifestation of SS, recently draws attention as a possible pathogenic mechanism of ocular symptoms and dry eye induction. The diagnostic criteria of SS changed recently, proposed by the American College of Rheumatology/Sjögren's International Collaborative Clinical Alliance (ACR/SICCA) in 2012, and the ocular surface staining score is the only required test for ocular manifestation of SS. However, other diagnostic methods evaluating tear film status, though excluded from the new criteria, are still important for the staging and treatment planning, including direct observation of tear film, tear film break up time, Schirmer test, and measurement of the tear film levels of inflammatory mediators. Eye-specific symptoms and signs and ocular treatment options for SS including tear substitutes, secretogogue, topical anti-inflammatory therapy with corticosteroids and cyclosporine, punctal occlusion, autologous serum, and mucolytic therapy were summarized and discussed in this review article.