L. Gargiulo, F. Cutrupi, Francisco Esoin, L. Melzi, Benedetta Storti, Nicola Rifino, Anna Bersano, S. Bianchi Marzoli
2026.2.3Case Reports in Ophthalmology
tlooto Summary
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.
Abstract
Introduction Inflammatory cerebral amyloid angiopathy (CAAri) is a rare condition characterized by vascular beta-amyloid deposition with secondary inflammation. Ocular involvement has been only sporadically described, but given the retina’s embryologic and microvascular similarities to the brain, retinal manifestations may provide a peripheral biomarker of disease activity. Case Presentation We report a 67-year-old woman with CAAri who developed recurrent hemorrhagic-inflammatory retinal vasculopathy during corticosteroid tapering. Multimodal retinal imaging revealed optic disc edema and peripapillary hemorrhages, while cerebral MRI lesions remained stable. Intensified immunosuppression with corticosteroids and cyclophosphamide led to complete and repeated resolution of retinal findings, despite unchanged neuroradiological features. Conclusion This case highlights the retina as a sensitive, dynamic marker of treatment response in CAAri. Multimodal retinal imaging, particularly OCT and angiography, may provide a valuable non-invasive tool for monitoring disease activity, even when cerebral lesions remain static.
Citation format
GARGIULO, L., et al. Retinal vasculopathy in inflammatory cerebral amyloid angiopathy: A case report of peripheral marker of treatment response. Case Reports in Ophthalmology, 2026, 17(1): 254–262.