Long-term Observation of Chinese Idiopathic Retinitis, Vasculitis, Aneurysms, and Neuroretinits (IRVAN) Patients and suggestion of a Revised Staging System

Abstract Background To investigate the clinical characteristics and intervention effects on Chinese patients with idiopathic retinitis, vasculitis, aneurysms, and neuroretinitis (IRVAN).Methods The consecutive series patients received eye examinations including fundus fluorescein angiography (FFA), optical coherence tomography (OCT) and systemic testing. Laser, including pan-retinal photocoagulation (PRP), oral corticosteroid and Pars plana vitrectomy (PPV) were used during the investigation period.Results Forty-two eyes of 21 patients aged 15-58 years old, 19 females and 2 males, initial decimal BCVA NP ~1.5 (0.55±0.38), were included. Eighteen eyes reached Stage 2; 21 eyes with neovascularization of disc or elsewhere and/or vitreous hemorrhage (VH) reached Stage 3; one eye had neovascular glaucoma (NVG) at Stage 5. Two eyes had proliferative vitroretinopathy (PVR). Thirty-four eyes of 20 patients of stage 2 and 3 accepted retinal photocoagulation. 27 eyes completed PRP. PPV was performed for 3 eyes at the first visit due to VH or PVR. Three eyes developed to Stage 3 from Stage 2. One eye of Stage 3 developed to PDR with retinal detachment. Additional 5 eyes, 3 heavy VH, 1 PVR and 1 macular epiretinal membrane eye, received PPV during the follow-up. Intra-retinal microvascular abnormality (IRMA) was found in 7 eyes. Aneurysms on the optic nerve head and artery bifurcations disappeared in 8 eyes and decreased in number in 2 eyes after one year of photocoagulation. Seven eyes were found with BCVA ≤0.1, of which 3 experienced PVR, 2 exudative maculopathy, 1 acute macular neuroretinopathy (AMN) eye and 1 eye due to NVG. The BCVA of the last visit was NLP~1.2 (0.53±0.38), no significant difference was found with initial BCVA.Conclusions Female is more susceptible to IRVAN. IRMA and AMN are firstly described in IRVAN patients. PVR and exudative maculopathy, the major causes for severe visual impairment, are suggested in the revised staging system.