Retinal Vasculopathy in Inflammatory Cerebral Amyloid Angiopathy: A Case Report of Peripheral Marker of Treatment Response

Inflammatory cerebral amyloid angiopathy (CAA-ri) is a rare immune response to vascular amyloid-β. Ocular involvement is exceptional, and the retina may represent an accessible biomarker of disease activity.

A 67-year-old woman with probable CAA-ri developed recurrent floaters and transient visual obscurations during steroid tapering, with bilateral optic disc edema and peripapillary hemorrhages. Multimodal retinal imaging supported an inflammatory hemorrhagic retinal arteriolar microangiopathy (normal CSF opening pressure; TREX1 negative). Retinal findings resolved after intensified immunosuppression (steroids plus mycophenolate), recurred during tapering, and resolved again after switching to cyclophosphamide with steroid escalation; brain MRI remained largely unchanged and brain biopsy later confirmed inflammatory CAA.

Multimodal retinal imaging may provide a dynamic, sensitive peripheral biomarker to monitor disease activity and treatment response in CAA-ri, even when cerebral MRI appears stable.