Purpose: The authors describe a 68-year-old woman in whom ischemic cho
roidopathy and optic neuropathy developed in association with primary
Sjogren's syndrome with central nervous system involvement. Methods: D
iagnosis of primary Sjogren's syndrome was made upon the association o
f keratoconjunctivitis sicca, minor salivary gland biopsy and serologi
c abnormalities. Fluorescein angiography showed signs highly suggestiv
e of ischemic choroidopathy. The authors discuss differential diagnosi
s and pathophysiology of such choroidal manifestations. Treatment cons
isted in massive steroid therapy. Secondarily, an immunosuppressant ag
ent (azathioprine) was successfully added because of neurological recu
rrences. Conclusion: Primary Sjogren's syndrome should be considered i
n the differential diagnosis of ischemic choroidopathy.