We report two cases of antiphospholipid antibody syndrome with elevated anti-beta 2-glycoprotein I antibodies. Patient 1 had systemic lupus erythematosus (SLE) and was treated with an oral corticosteroid and aspirin. There were foci of sheathed retinal arterioles appearing as white lines in the fundus of both eyes. The sheathed arterioles of the peripheral fundus of the right eye were completely or incompletely occluded. Recanalization occurred during a 10 month follow-up. Patient 2 had hypertension and multiple brain infarction but no association with collagen diseases. Major retinal arterioles showed sheathing. Fluorescein angiography demonstrated progressive occlusion or stenosis of these major arterioles with extensive insufficiency of regional capillary bed circulation. Retinal photocoagulation was applied to both eyes which developed neovascularization. Vitrectomy was performed in the left eye with recurrent vitreous hemorrhage. Antiphospholipid antibody syndrome should be considered in cases of sheathed or white retinal arterioles.

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