Presentation, aetiology and outcomes of corneal ulceration in Sjogren's Syndrome

To report the clinical course of corneal ulceration/perforation in patients with Sjogren's syndrome.

Retrospective descriptive study of patients diagnosed with Sjogren's syndrome (primary and secondary) and corneal ulceration over past 8 years at tertiary eye care network. Assessed parameters were demographics, clinical details, microbiological profile, types of intervention and their outcomes.

Forty-six eyes of 44 patients (11 males; mean age, 50 years) had corneal ulceration (28 eyes) or perforation (18 eyes) at the time of presentation. Of 46 eyes, 38 had sterile ulceration/perforation and rest 8 showed microorganism on microscopy or culture. The location was peripheral in 63% of eyes and it was the first presenting sign of Sjogren's syndrome in 26% of patients. All 43 eyes (3 lost to follow-up after intervention) had successful management of corneal ulceration/perforation. Twenty-eight eyes with ulceration required medical management alone in 15 eyes, soft contact lens and isobutylcyanoacrylate in 12 eyes, and amniotic membrane grafting in one eye. Four eyes with ulceration worsened and required penetrating keratoplasty (n = 2), and amniotic membrane grafting (n = 2). Corneal perforations were successfully managed with isobutylcyanoacrylate patch and BCL (n = 15), corneal patch graft (n = 2) and multilayered amniotic membrane grafting (n = 1). The average time taken for ulcers to heal was 49 days over a mean follow-up duration of 10 months.

Corneal ulceration or perforation in Sjogren's syndrome is often sterile and can be a presenting sign of undiagnosed SS. These patients usually respond to intensive medical therapy and bandage contact lens and isobutylcyanoacrylate patch application.