Researchers Identify Risk Factors for Severe Visual Impairment in Sjögren Syndrome

A very serious case of a corneal ulcer with hypopyon, caused by a bacterial infection. A hypopyon is a collection of pus in the anterior chamber of the eye. (Photo by © Dr. Charles J. Ball/CORBIS/Corbis via Getty Images)
Researchers investigate risk factors for severe visual impairment associated with the systemic disease.

Rheumatoid arthritis (RA) is the leading risk factor for various types of ocular decline in secondary Sjögren syndrome (SS), according to research results published in the American Journal of Ophthalmology. These include severe visual impairment (SVI) and corneal complications. 

Researchers conducted a retrospective case series, reviewing the ocular data of 919 consecutive patients with SS who presented to a network of eye centers and were diagnosed in an 8-year period. The outcome measures were the risk factors associated with SVI, as well as vision-threatening corneal complications at presentation.

SS is an autoimmune disorder that affects exocrine glands and systemic organs, the study explains. The disease is divided into primary and secondary etiopathogenic groups, due to their differences in clinical presentation, autoantibodies, and immunological makeup. One ophthalmic feature of this disease is aqueous deficient dry eye (ADDE) due to the destruction of lacrimal glands. Corneal complications, such as ulceration and corneal melting, which the researchers say is found in 3% to 4% of SS patients, were also noted..

Though previous research had been done on ocular symptoms in patients with SS, the researchers explain that their risk factors had not been studied before. Of the 919 patients who met the criteria for diagnosis of SS and whose data was evaluated, 285 had primary SS and 634 had secondary SS. 

Among patients with secondary SS, diseases present were RA in 98.1% (622/634), lupus erythematosus in 0.79% (5/634), psoriasis in 0.79% (5/634), and scleroderma in 0.6% (4/634). In terms of all SS patients whose data was evaluated, corneal scarring was more present in eyes with SVI (n=185) compared with those with no SVI (36.2% vs 13.6%), as well as corneal ulceration (13% vs 1.3%), and glaucoma (3.7% vs .4%). Other statistically significant risk factors included lesser Schimer values, increasing age at diagnosis, and cataract. Among eyes with and without SVI, the distribution of primary and secondary SS was similar. 

As for corneal ulceration and perforation, 46 eyes of the 1838 analyzed (2.5%) had one of these conditions at first presentation to the institute. Microbial keratitis was present in 31 of these eyes (67.3%), and the number of eyes with sterile ulceration was 15 (32.6%). Overall, the statistically significant risk factors for corneal complications were scleritis (P <.0001) and secondary SS (P <.009).

The researchers say that strengths of the study were the large sample size and wide geographic range of the data analyzed. Study limitations include missing OSDI scores in many patients as well as follow-up data.

“A prospective longitudinal cohort study would be ideal to identify the true incidence and validate the risk factors of severe visual impairment and corneal complications in patients with SS,” the study concludes.


Singh S, Das AV, Basu S. Ocular involvement in Sjogren’s syndrome: risk factors for severe visual impairment and vision-threatening corneal complications. Am J Ophthalmol. Published online December 29, 2020. doi:10.1016/j.ajo.2020.12.019.