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  • Sjögren Syndrome and Risk of Visual and Corneal Problems

    By Lynda Seminara
    Selected By: Richard K. Parrish II, MD
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    Journal Highlights

    American Journal of Ophthalmology, May 2021

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    Singh et al. explored risk factors for severe visual impairment (VI) and corneal complications among patients with Sjögren syndrome. They found that severe VI was more common with older age, corneal involvement, lower Schirmer test scores, and the presence of glaucoma or cataract. Scleritis was the strongest risk factor for corneal perforation or ulceration.

    In this study, the authors analyzed data for patients with primary or sec­ondary Sjögren treated consecutively during an eight-year period. They con­ducted multivariate analyses and calcu­lated odds ratios (OR) to determine the greatest baseline risk factors for severe VI (defined as BCVA better than 20/200) and for vision-threaten­ing corneal complications (defined as ulceration or perforation).

    The study population included 919 patients (1,838 eyes). Of these, 285 pa­tients (31%) had primary Sjögren, and 634 (69%) had secondary disease. Most were female (82.2%), and the median age was 50 years.

    Cases of Sjögren were diagnosed according to criteria of the American College of Rheumatology. The most common cause of secondary disease was rheumatoid arthritis (98.1%), followed by systemic lupus erythema­tosus (0.79%), psoriasis (0.79%), and scleroderma (0.6%).

    Ten percent of eyes had severe VI at presentation, and 2.5% had corneal complications at that time. Baseline factors found to independently increase the risk of severe VI were the presence of corneal scarring (p < .00001; OR, 3.00) or corneal ulceration (p < .00001; OR, 12.96), low Schirmer values (p = .0084; OR, 0.93), concurrent cataract (p = .0036; OR, 2.4) or glaucoma (p = .04; OR, 4.09), and age older than 40 years (p = .005; OR, 1.02).

    The greatest risk factors for corneal complications were presence of scleritis (p < .0001, OR, 8.9) and diagnosis of secondary Sjögren (p = .009; OR, 2.94). Among eyes with both scleritis and corneal ulceration, the ulcer was central in two eyes and peripheral in five eyes. Retinal pathology was more common with primary Sjögren (p = .03) and consisted mostly of macular degener­ation and hypertensive changes. There was one case of low-grade non-Hodgkin orbital lymphoma, which occurred in a patient with primary Sjögren.

    The original article can be found here.