Primary Sjögren's syndrome is frequently characterized by a sicca syndrome without associated connective tissue disease. Association with an optic neuropathy is uncommon.
We report a case of optic neuropathy in a 59-year-old woman known to have primary Sjögren's syndrome confirmed clinically and histologically 2 years ago. She suddenly presented an initial bilateral visual loss. The ophthalmological exam noted a visual acuity of 1/10 in the right eye and limited to light perception in the left eye, with bilateral optic ischemic neuropathy more developed in the left eye. Fluorescein angiography showed, signs of ischemic neuropathy. The diagnosis of Horton disease was suspected, but subnormal blood velocity and a negative biopsy of the temporal artery confirmed the diagnosis of optic neuropathy associated with primary Sjögren's syndrome. General steroid therapy improved optic neuropathy in the right eye but was ineffective in the left eye.
Optic neuropathy associated with Sjögren's syndrome is rare but must be considered the most common ophthalmological manifestation of the disease. Visual prognosis depends on the rapidity of diagnosis and therapy.