Corneal complications of Sjögren's disease: Insights from a large tertiary ophthalmological care center.

PURPOSE: To report the demographic characteristics, clinical spectrum, and outcomes of patients with extra-glandular corneal manifestations of Sjögren's disease (SjD). DESIGN: Retrospective review of electronic medical records. PARTICIPANTS: Consecutive patients with SjD diagnosed per the 2016 American College of Rheumatology/European League Against Rheumatism criteria at a tertiary ophthalmology center. METHODS: Patients were systematically evaluated, diagnosed, and followed longitudinally. A database was generated by searching electronic records, collecting data on demographics, clinical and laboratory findings, treatments, and outcomes. MAIN OUTCOME MEASURES: Prevalence and management of extra-glandular corneal manifestations of SjD. RESULTS: A total of 415 patients were included, mostly female (92.5 %), with a mean age of 63 years (range: 18-91) and a mean follow-up of 34 months (range: 1-120). Standalone SjD was present in 126 patients (30.28 %), and 289 (69.63 %) had associated autoimmune diseases, most commonly rheumatoid arthritis (n = 221) and systemic lupus erythematosus (n = 26). Diagnosis was based on ACR/EULAR score-based criteria (score ≥4) with positive serology (n = 372, 89.63 %) and positive minor salivary gland biopsy (n = 46, 11.08 %). Corneal complications were observed in 113 patients (27.2 %), including scarring (38/113), epithelial defects (22/113), and perforation. Corneal perforation occurred in 53 patients (12.77 %), most commonly at presentation (47/53, 88.67 %). Perforation rates were 5.56 % (7/126) in standalone SjD and 15.92 % (46/289) in SjD with other autoimmune diseases. At presentation, 56.6 % of patients with perforation (30/53) were not on systemic immunomodulatory treatment (IMT); they were subsequently treated. Six patients (11.32 %) developed perforation during follow-up while already on IMT and required escalation. All six had coexisting autoimmune diseases, primarily rheumatoid arthritis. To manage complications, 75 surgical procedures were performed, including keratoplasty. Five eyes were enucleated or eviscerated due to delayed presentation and infection. CONCLUSIONS: Corneal complications, including perforation, are common in SjD and may occur even in the absence of other autoimmune diseases. Many patients present without systemic treatment. These findings support incorporating corneal manifestations into SjD disease activity indices to enable earlier medical intervention alongside surgical care.

Duke Scholars

Author Esen Karamursel Akpek Ophthalmology, Vitreoretinal Diseases & Surgery