Ocular Involvement in Sjögren Syndrome: Risk Factors for Severe Visual Impairment and Vision-Threatening Corneal Complications

Abstract

To assess the risk factors for severe visual impairment (SVI) and corneal complications in primary and secondary Sjögren syndrome (SS). Retrospective case series. Ocular data of all consecutive SS patients presenting to an eye-care network and receiving a diagnosis according to 2012 American College of Rheumatology criteria over the past 8 years were reviewed. risk factors associated with SVI (best-corrected visual acuity <20/200) and vision-threatening corneal complications (ulceration or perforation) at presentation were evaluated using multivariate analysis and odds ratios (OR). Of the 919 patients, 285 (31%) had primary and 634 (69%) had secondary SS. The most common cause of secondary SS was rheumatoid arthritis (98.1%), followed by systemic lupus erythematosus (0.79%), psoriasis (0.79%), and scleroderma (0.6%). Among the 1,838 eyes, SVI was noted in 10%, and 2.5% had corneal complications at presentation. The presence of corneal scarring (P < .00001; OR: 3.00), corneal ulceration (P < .00001; OR: 12.96), low Schirmer values (P= .0084; OR: 0.93), cataract (P= .0036; OR: 2.4), glaucoma (P= .04; OR: 4.09), and age at diagnosis (P= .005; OR: 1.02) were independent risk factors for developing SVI. The risk factors for corneal complications were presence of scleritis (P < .0001; OR: 8.9) and a diagnosis of secondary SS (P= .009; OR: 2.94). In patients with SS, severity of dryness, corneal ulceration and scarring, cataract, and glaucoma are factors associated with poor visual acuity. Eyes with scleritis have a greater risk of developing vision-threatening corneal complications and therefore should be monitored closely.