IRVAN syndrome
ORPHA:209943· ICD-10 H35.0
Definition
A rare retinal vasculopathy disease characterized by idiopathic retinal vasculitis (IRV), aneurysmal dilations (A) at arteriolar bifurcations, and neuroretinitis (N), which if untreated progresses to peripheral capillary non-perfusion, retinal neovascularization, and macular exudation, leading to severe, bilateral vision loss.
- Prevalence
- <1 / 1 000 000
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood