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

Abstract

Purpose: To assess the risk factors for severe visual impairment (SVI) and corneal complications in primary and secondary Sjögren syndrome (SS).

Design: Retrospective case series.

Methods: 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.

Main outcome measurement: 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).

Results: 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).

Conclusions: 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.