Background: Optic neuritis (ON) is often the first symptom of multiple sclerosis (MS) and neuromyelitis optica (NMO) while there has been very little research reported on ON combined with Sjögren's syndrome (SS). The aim of this study is to provide different treatments and services for and NMO patients combined with SS.

Methods: Twenty-seven patients with ON combined SS were divided into two groups: corticosteroid group (C group, methylprednisolone sodium succinate, 14 patients) and corticosteroid+ immunosuppressant group (C+I group, leflunomide, 13 patients). ON relapse times in 1 year after treatment, number of patients who relapsed to NMO/MS in 1 years, visual acuity and retina nerve fiber layer (RNFL) thickness were measured. Mann Whitney-Wilcoxon test was used to compare continuous variables and Chi-square test or Fisher's exact test was to compare proportions.

Results: ON combined with SS patients had higher incidence rates in middle-aged women who have binocular damage and heavier visual function damage or when there is an easy relapse, and the patients are often hormone dependent. The patients are more likely anti-aquaporin-4 IgG seropositive (70.4%). They are liable to form a centrocecal scotoma and tubular vision. The times of relapse decreased in patients who used immunosuppressant, and a significant difference was found between immunosuppressant and non-immunosuppressant groups in visual acuity recovery during 6-month follow-up period (P < 0.05); however, the RNFL thickness at the four quadrants was not significantly different.

Conclusions: The effect of immunosuppressant plus corticosteroid on the early onset of ON combined with SS was to provide ON remedy and to prevent recurrence in clinics. This study provides a significant reference for the prevention and treatment of ON on the basis of immunosuppressant and corticosteroid.

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