Central Retinal Artery Occlusion in Churg-Strauss Syndrome

Purpose: Churg-Strauss syndrome (CSS) is a rare form of small vessel necrotizing vasculitis. Retinal artery and vein occlusion are very rare findings in these patients and they are treated by high-dose corticosteroids and cytotoxics. We describe a rare case of CSS under high-dose corticosteroids and cytotoxic treatment, presenting central retinal artery occlusion (CRAO). Case report: A 45-year-old man with a history of CSS, who had been admitted in the rheumatology ward to receive high-dose pulse corticosteroids, was presented with sudden loss of vision in his left eye four days after admission. He was receiving high doses of oral and intravenous prednisolone and a pulse of cyclophosphamide. Visual acuity (VA) was poor light perception and relative afferent papillary defect was present (3-4 +). A CRAO was diagnosed by the funduscopic appearance of retinal whitening, macular thinning, and mild optic disc paleness. In fluorescein angiogram, retinal filling and normal choroidal filling were absent. Conclusion: CSS-associated CRAO should be considered when acute visual loss occurs. Although taking corticosteroids and cytotoxics is the main treatment of patients with CCS, patients may progress to CRAO under this treatment.