Optic neuropathy as an initial manifestation of Sjögren's syndrome

Although optic neuropathy is a known complication of systemic lupus erythematosus, its association with other connective tissue diseases is less well documented. We describe 3 previously asymptomatic women presenting with optic neuropathy in whom changes highly suggestive of primary Sjögren's syndrome (SS) were found by minor salivary gland biopsy 1-6 years after initial presentation. Typical symptoms of SS were absent initially and developed slowly, while serologic abnormalities were consistently present. Optic neuropathy may be a presenting neurologic feature of SS and may antedate typical signs and symptoms of SS. Primary SS should be considered in the differential diagnosis of optic neuropathy.