Abstract: Objective To investigate the systemic condition, fundus characteristics, treatment methods and prognosis of progressive outer retinal necrosis (PORN) with acquired immunodeficiency syndrome (AIDS). Design Retrospective case series. Participants 6 cases (11 eyes) of PORN with AIDS in Beijing You'an Hospital from 2015 to 2019. Methods Visual acuity, fundus, optical coherence tomography (OCT) and varicella zoster virus (VZV)-DNA, CD4+ T lymphocytes in blood and human immunodeficiency virus (HIV) viral load were analyzed. 3 eyes underwent intravitreal injection of ganciclovir and (or) phosphonate combined with systemic therapy. The follow-up was for 3~26 months. Main Outcome Measures Fundus changes of AIDS combined with PORN, VZV-DNA values in aqueous humor, fundus changes after intravitreal injection, peripheral blood CD4+ T lymphocyte count, and HIV viral load. Results The 6 patients were all males, average age 27.6±3.2 years old. 9 eyes (81.8%) had a best corrected visual acuity of ≤ 0.2 at the first diagnosis. 11 eyes showed painless vision loss. The fundus showed progressive peripheral circular retinal necrosis, yellow-white necrotic foci with clearer multifocal borders progressed concentrically, no or slight vitreous reaction, 6 eyes with exudative retinal detachment. Early lesions on OCT only affect the outer layer of the retina, and later the outer layer becomes thinner. The levels of VZV-DNA in the aqueous humor of the 11 eyes were all positive. CD4+ T lymphocyte count in peripheral blood of patients was average (10.8±2.5)/μl. The HIV viral load was average (246289±462.7) copies/ml. The treatment period of combined with systemic antiviral therapy, intravitreal injection of ganciclovir and (or) sodium phosphonate in 3 eyes was 72~123 days, and their fundus lesions were controlled and vision was improved (1 eye recovered from light perception to hand movement, 1 eye returned to 0.1 from count fingers, and 1 eye remained at 0.5). Conclusion PORN is the most serious eye opportunistic infection in AIDS patients; the fundus shows early lesions involving the outer layer of the retina, no or slight anterior chamber and vitreous reaction. Compared with other AIDS opportunistic eye infections, PORN progresses faster and has a poor prognosis. Viral nucleic acid detection in the eye fluid can help diagnosis. （Ophthalmol CHN, 2020, 29: 375-379）

Key words: progressive outer retinal necrosis, acquired immunodeficiency syndrome, varicella zoster virus