The authors describe a 68-year-old woman in whom ischemic choroidopathy and optic neuropathy developed in association with primary Sjögren's syndrome with central nervous system involvement.
Diagnosis of primary Sjögren's syndrome was made upon the association of keratoconjunctivitis sicca, minor salivary gland biopsy and serologic abnormalities. Fluorescein angiography showed signs highly suggestive of ischemic choroidopathy. The authors discuss differential diagnosis and pathophysiology of such choroidal manifestations. Treatment consisted in massive steroid therapy. Secondarily, an immunosuppressant agent (azathioprine) was successfully added because of neurological recurrences.
Primary Sjögren's syndrome should be considered in the differential diagnosis of ischemic choroidopathy.