When vision loss signals vasculitis: central retinal artery occlusion leading to microscopic polyangiitis diagnosis-a...

Central retinal artery occlusion (CRAO) is an ophthalmic emergency characterized by sudden vision loss; it is rarely associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Herein, we report a case of a man in his 80s who, experiencing persistent fever, weight loss, and myalgia, received corticosteroid therapy at a local hospital for a presumptive diagnosis of polymyalgia rheumatica. While on this treatment, he suddenly developed vision loss in the left eye; visual acuity was limited to light perception, and fundus examination revealed a cherry-red spot in the macula, consistent with CRAO. The patient was urgently referred and admitted to the rheumatology department of our hospital for evaluation and management of suspected systemic vasculitis underlying CRAO. The presence of persistent fever, elevated inflammatory markers, positive myeloperoxidase-ANCA, interstitial lung disease, purpura, and small-vessel vasculitis confirmed via muscle biopsy led to the diagnosis of microscopic polyangiitis. Given this clinical course and definitive diagnosis, his initial systemic symptoms were considered early manifestations of the underlying microscopic polyangiitis. The patient was treated with methylprednisolone pulse therapy and rituximab, followed by azathioprine; the inflammatory markers improved, and visual acuity recovered to hand motion by discharge. This case highlights that when CRAO occurs alongside systemic symptoms, ANCA-associated vasculitis should be strongly considered as a potential underlying cause. Timely identification of such systemic vasculitis is crucial to enhance the possibility of visual recovery and to reduce complications affecting vital organs beyond the eye.