• Mai Takagi, Takatoshi Kobayashi, Teruyo Kida, Nanae Takai, Hiromi Shoda, Koichi Maruyama, Rei Tada, Shigeki Makino, and Tsunehiko Ikeda.
• Department of Ophthalmology, Osaka Medical College.
• Medicine (Baltimore). 2020 Aug 28; 99 (35): e21934.

RationaleWe report a case of central retinal artery occlusion (CRAO) accompanied by choroidal folds in a patient positive for myeloperoxidase (MPO)-antineutrophil cytoplasmic antibody (ANCA).Patient ConcernsThe study involved a 67-year-old female patient who presented at the Department of Ophthalmology, Osaka Medical College, Takatsuki-City, Osaka, Japan on October 24, 2016 after becoming aware of a sudden decrease of visual acuity (VA) in her right eye. Other than suffering with scleritis 6-months previous, there was no obvious past history.DiagnosisUpon examination, the VA in her right eye was hand motion, and the anterior segment of that eye showed thinning of the superior sclera. Macular edema in the inner retina and cherry red spots were observed in the ocular fundus, and optical coherence tomography (OCT) findings showed hyperreflectivity of the inner retina and choroidal folds. Fluorescein angiography (FA) examination of the fundus showed scattered areas of no retinal perfusion, and indocyanine green angiography (IA) findings of the fundus indicated a possible choroidal circulatory disturbance in her right eye. Blood test findings revealed the patient to be positive for MPO-ANCA. Based on the above findings, the patient was diagnosed with CRAO and choroidal circulatory disturbance due to ANCA-associated vasculitis.InterventionsFor treatment, steroid semi-pulse therapy was initiated.OutcomesPost treatment initiation, the fundus features and choroidal folds gradually improved, and her VA slightly improved to 0.08.LessonsBased on the FA, IA, and OCT findings, the present case was considered to have CRAO accompanied by choroidal circulatory disturbance due to ANCA-associated vasculitis, a rare disease that may be complicated by choroidal circulatory disturbances.

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