A Case of Primary Sjögren's Syndrome Presenting Various Visual Field Changes

Clinical course and visual field changes were followed in a 43-year-old woman who was diagnosed with primary Sjögren's syndrome. She visited our institute for an inferior visual field defect in the right eye. At the initial examination, inferior altitudinal hemianopia was found in her right eye. At 22 days after onset, she had superior altitudinal hemianopia instead of inferior altitudinal hemianopia. Her right eye lost light perception once thereafter, but her visual acuity recovered to 20/40 on intravenous methylprednisolone. Temporal hemianopia still remained in the right eye. The level of autoantibody against SS-A peptide was higher than the upper limit of measurement, while that against SS-B peptide was within normal range. She was negative for three antineuronal antibodies (anti-Yo, Hu, and Ri antibodies). Sjögren's syndrome often presents various neurologic manifestations including optic neuropathy. Although little is known about the pathogenesis of SS-associated optic neuropathy, ischemia secondary to small-vessel vasculitis may play an important role in neurologic disturbances in the presence of an increased level of anti-SS-A antibody. Anti-SS-A antibody may participate in optic neuropathy through the small-vessel vasculitis.