Bilateral simultaneous central retinal artery occlusions in wegener granulomatosis

A 46-year old woman developed simultaneous central retinal artery occlusions (CRAOs) in Wegener granulomatosis (WG). She had presented six years earlier with xerostomia, skin rash, and arthralgias and received a diagnosis of Sjogren syndrome. Anti-neutrophilic cytoplasmic antibody (ANCA) was negative. Months prior to the CRAOs, she had developed hearing loss, proptosis, and scleritis that were not responsive to prednisone 50 mg/d. The CRAOs occurred while she was being treated at this dose level. ANCA was now positive. This is the 12th case of CRAO in WG and the 6th case of bilateral CRAOs reported in the English literature. It emphasizes that serious irreversible visual complications may occur even when the patient is being treated with substantial corticosteroid doses.