Diagnostic Challenges in an Atypical Case of IRVAN Syndrome: Literature Review

A 60-year-old female with known seropositive rheumatoid arthritis and other co-morbids, presented with right eye hypertensive retinopathy and exudative macroaneurysms. Over the years, she developed vitreous haemorrhage, macula oedema and full thickness macula hole. Fluorescein angiography showed macroaneurysms and ischaemic retinal vasculitis. Initial diagnosis was thought to be hypertensive retinopathy with macroaneurysms and retinal vasculitis secondary to rheumatoid arthritis. Laboratory investigations did not support other causes for macroaneurysms and vasculitis. Hence, delayed diagnosis of IRVAN syndrome was made after detailed review of clinical findings, investigations, and angiographic evidence. Our understanding of IRVAN continues to evolve in the face of challenging presentations. To our knowledge, this is the first reported case of IRVAN associated with rheumatoid arthritis.